Nevoid basal cell carcinoma syndrome associated with renal cysts and hypertension

A case of nevoid basal cell carcinoma syndrome with a coincidental finding is presented. In addition to the odontogenic cysts, hypertelorism, scapula deformity, rib fusion, dural calcification, and short fourth metacarpal bones of the syndrome, the patient had cystic disease of both kidneys with a large cyst of the right kidney. This case also illustrates that the presence of painful jaw lesions may lead the patient with this syndrome to seek treatment initially.