Abstract: Report of one case of nevoid basal cell carcinoma syndrome.A 44 years old male patient with facial painless black rash for more than 1 year came to our department.Dermatological examination:maculas and papules on the face and around the ear,railiary to bean-size,black and brown,well-demarcated,some with roughness surface and uneven pigment distribution,slightly uplift.Visible scattered needle-sized retraction was seen in the palms and toes.Pathological examination showed basal cell carcinoma.Diagnosis:nevoid basal cell carcinoma syndrome.

Key words: Nevus, Basal cell carcinoma, Syndrome, Papule