We reported a typical case of nevoid basal cell epithelioma syndrome (NBCES) . 40 year-old man, no similar disease and no consanguinity of his family history. He had large ulcerated black lesion on the r-cheek and multiple black plaques of basal cell epithelioma on the trunk and extremities. He had all the five symptoms, i.e. multiple basal cell epithelioma, jaw cysts, keratotic pits on the palm and sole, ectopic calcification and skeletal abnormalities. Diagnostic criteria of NBCES was still confused and unclear. We considered to regard the five symptoms as important for diagnosis of NBCES.