EEC syndrome is a rare autosomal dominant disorder, characterized by absence of fingers and toes, ectodermal dysplasia and cleft lip or cleft palate. Other features could be associated with the disease, such as abnormalities of the genitourinary system, conductive or sensorineural hearing loss, choanal atresia, mammary gland/nipple hypoplasia, ophthalmological findings, gland abnormalities and in rare cases, presence of a white sponge nevus, delayed developmental milestones, and malignant lymphoma.