-Major criteria include radiographic triad of 1) odontogenic keratocysts, 2)calcification of the falx, 3) Rib abnormalities including bifid, fused andd markedly splayed ribs.

-Other major criteria include as well as two or more basal cell carcinomas in a patient less than 20 years old, a first degree relative with First-degree relative with Gorlin syndrome,and three or more palmar or plantar pits.

Autosomal dominant

Propensity of these patients to develop multiple neoplasms, including basal cell carcinomas and meduloblastoma

The genetic locus for Gorlin syndrome is 9q22.3-q31, as determined with linkage analysisGermline mutations in the patched gene have been found in patients with Gorlin syndrome and are thought to cause the disorder. PTCH is a tumor suppressor gene. More than 50 germline mutations are described. About 40% of cases of Gorlin syndrome represent new mutations.

-Minor criteria include: Macrocephaly, Congenital malformations (eg, cleft lip or palate, frontal bossing, coarse face, hypertelorism), other skeletal abnormalities (eg, Sprengel deformity, marked pectus deformity, or syndactyly of the digits, Radiologic abnormalities (eg, bridging of the sella turcica, vertebral anomalies [eg, hemivertebrae, fusion or elongation of the vertebral bodies], modeling defects of the hands and feet, or flame-shaped lucencies of the hands or feet, Ovarian fibroma and Medulloblastoma.