| Q80 | Congenital ichthyosis |
| Q80.0 | Ichthyosis vulgaris |
| Q80.1 | X-linked ichthyosis |
| Q80.2 | Lamellar ichthyosis |
| Q80.3 | Congenital bullous ichthyosiform erythroderma |
| Q80.4 | Harlequin fetus |
| Q80.8 | Other congenital ichthyosis |
| Q80.9 | Congenital ichthyosis, unspecified |
| Q81 | Epidermolysis bullosa |
| Q81.0 | Epidermolysis bullosa simplex |
| Q81.1 | Epidermolysis bullosa letalis |
| Q81.2 | Epidermolysis bullosa dystrophica |
| Q81.8 | Other epidermolysis bullosa |
| Q81.9 | Epidermolysis bullosa, unspecified |
| Q82 | Other congenital malformations of skin |
| Q82.0 | Hereditary lymphedema |
| Q82.1 | Xeroderma pigmentosum |
| Q82.2 | Mastocytosis |
| Q82.3 | Incontinentia pigmenti |
| Q82.4 | Ectodermal dysplasia (anhidrotic) |
| Q82.5 | Congenital non-neoplastic nevus |
| Q82.8 | Other specified congenital malformations of skin |
| Q82.9 | Congenital malformation of skin, unspecified |
| Q83 | Congenital malformations of breast |
| Q83.0 | Congenital absence of breast with absent nipple |
| Q83.1 | Accessory breast |
| Q83.2 | Absent nipple |
| Q83.3 | Accessory nipple |
| Q83.8 | Other congenital malformations of breast |
| Q83.9 | Congenital malformation of breast, unspecified |
| Q84 | Other congenital malformations of integument |
| Q84.0 | Congenital alopecia |
| Q84.1 | Congenital morphological disturbances of hair, not elsewhere classified |
| Q84.2 | Other congenital malformations of hair |
| Q84.3 | Anonychia |
| Q84.4 | Congenital leukonychia |
| Q84.5 | Enlarged and hypertrophic nails |
| Q84.6 | Other congenital malformations of nails |
| Q84.8 | Other specified congenital malformations of integument |
| Q84.9 | Congenital malformation of integument, unspecified |
| Q85 | Phakomatoses, not elsewhere classified |
| Q85.0 | Neurofibromatosis (nonmalignant) |
| Q85.00 | Neurofibromatosis, unspecified |
| Q85.01 | Neurofibromatosis, type 1 |
| Q85.02 | Neurofibromatosis, type 2 |
| Q85.03 | Schwannomatosis |
| Q85.09 | Other neurofibromatosis |
| Q85.1 | Tuberous sclerosis |
| Q85.8 | Other phakomatoses, not elsewhere classified |
| Q85.9 | Phakomatosis, unspecified |
| Q86 | Congenital malformation syndromes due to known exogenous causes, not elsewhere classified |
| Q86.0 | Fetal alcohol syndrome (dysmorphic) |
| Q86.1 | Fetal hydantoin syndrome |
| Q86.2 | Dysmorphism due to warfarin |
| Q86.8 | Other congenital malformation syndromes due to known exogenous causes |
| Q87 | Other specified congenital malformation syndromes affecting multiple systems |
| Q87.0 | Congenital malformation syndromes predominantly affecting facial appearance |
| Q87.1 | Congenital malformation syndromes predominantly associated with short stature |
| Q87.2 | Congenital malformation syndromes predominantly involving limbs |
| Q87.3 | Congenital malformation syndromes involving early overgrowth |
| Q87.4 | Marfan's syndrome |
| Q87.40 | Marfan's syndrome, unspecified |
| Q87.41 | Marfan's syndrome with cardiovascular manifestations |
| Q87.410 | Marfan's syndrome with aortic dilation |
| Q87.418 | Marfan's syndrome with other cardiovascular manifestations |
| Q87.42 | Marfan's syndrome with ocular manifestations |
| Q87.43 | Marfan's syndrome with skeletal manifestation |
| Q87.5 | Other congenital malformation syndromes with other skeletal changes |
| Q87.8 | Other specified congenital malformation syndromes, not elsewhere classified |
| Q87.81 | Alport syndrome |
| Q87.89 | Other specified congenital malformation syndromes, not elsewhere classified |
| Q89 | Other congenital malformations, not elsewhere classified |
| Q89.0 | Congenital absence and malformations of spleen |
| Q89.01 | Asplenia (congenital) |
| Q89.09 | Congenital malformations of spleen |
| Q89.1 | Congenital malformations of adrenal gland |
| Q89.2 | Congenital malformations of other endocrine glands |
| Q89.3 | Situs inversus |
| Q89.4 | Conjoined twins |
| Q89.7 | Multiple congenital malformations, not elsewhere classified |
| Q89.8 | Other specified congenital malformations |
| Q89.9 | Congenital malformation, unspecified |