Keratinocytic epidermal nevus with oral involvement and cleft palate

Epidermal nevus syndromes are a heterogeneous group of genetic disorders characterized by the presence of an epidermal nevus with visceral involvement. Keratinocytic epidermal nevus syndromes are not a clear entity, but all the reported cases showed the presence of a keratinocytic epidermal nevus associated with different systemic lesions. In our report the keratinocytic epidermal nevus syndrome is defined by the presence of the nevus on the face, intraoral lesions and cleft palate in a 1-year-old girl