Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome, shows autosomal dominant pattern of inheritance with variable expressivity. The most important features are the occurrence of basal cell carcinoma and odontogenic keratosis. Recently, we experienced a case of nevoid basal cell carcinoma syndrome with multiple odontogenic keratocysts, basal cell carcinoma on cheek, cerebral calcification and palmar pits. We report this case with literature review.