Table 1.

Major Criteria

Minor Criteria

More than 2 BCCs or one under the age of 20 years


Odontogenic keratocyts of the jaw proven by histology

Congenital malformations: cleft lip or palate, frontal bossing, “coarse face”, moderate or severe hypertelorism

Three or more palmar or plantar pits

Other skeletal abnormalities: Sprengel deformity, marked pectus deformity, marked syndactyly of the digits

Bilamellar calcification of the falx cerebri

Radiological abnormalities: bridging of the sella turcica, vertebral anomalies such as hemivertebrae, fusion or elongation of the vertebral bodies, modeling defects of the hands and feet, or flame shaped lucencies of the hands or feet

Bifid, fused, or markedly splayed ribs

Ovarian fibroma

First degree relative with NBCC syndrome



Adapted from Kimonis, et al. [2] Diagnosis requires two major or one major and two minor criteria.