Abstract: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant hereditary disease with more than 100 clinical manifestations, whose main clinical manifestations are skin basal cell carcinoma, odontogenic keratocystic tumor (OKC), excessive keratosis, pitting of the palms and soles of the feet, skull abnormality and falciparum calcification, orbital spacing widening, facial deformity, giant brain deformity, cleft lip and palate, etc. A case of nevoid basal cell carcinoma syndrome is reported in this paper. The pathogenesis, incidence, clinical manifestation, diagnosis and treatment of the disease are discussed in combination with relevant literature.

Key words: Naevoid basal cell carcinoma syndrome, odontogenic keratocystic tumor, diagnosis, treatment