The study reviews the clinical and radiological findings in 105 individuals with Nevoid Basal Cell Carcinoma Syndrome (NBCC), also known as Gorlin syndrome. NBCC is an autosomal dominant disorder linked to chromosome 9q22.3-q31, caused by mutations in the *PTC* gene, which is the human homologue of the *Drosophila patched* gene. The syndrome includes multiple basal cell carcinomas (BCCs), keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies, and calcification of the falx cerebri. The study found that 80% of whites and 38% of African-Americans had at least one BCC, with the first tumor occurring at a mean age of 23 years for whites and 21 years for African-Americans. Jaw cysts were present in 74% of individuals, with the first tumor occurring in 80% by age 20 years. Palmar and plantar pits were observed in 87% and 81% of affected individuals, respectively. Ovarian fibromas were diagnosed in 17% of women, and medulloblastoma occurred in 4 patients. Physical findings included coarse face, relative macrocephaly, hypertelorism, frontal bossing, pectus deformity, and Spengel deformity. Radiological signs included calcification of the falx cerebri in 65%, tentorium cerebelli in 20%, bridged sella in 68%, bifid ribs in 26%, hemivertebrae in 15%, fusion of vertebral bodies in 10%, and flame-shaped lucencies in the hands and feet in 30%. The study discusses the diagnostic criteria for NBCC and provides recommendations for surveillance and management, emphasizing the importance of early detection and regular follow-up.The study reviews the clinical and radiological findings in 105 individuals with Nevoid Basal Cell Carcinoma Syndrome (NBCC), also known as Gorlin syndrome. NBCC is an autosomal dominant disorder linked to chromosome 9q22.3-q31, caused by mutations in the *PTC* gene, which is the human homologue of the *Drosophila patched* gene. The syndrome includes multiple basal cell carcinomas (BCCs), keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies, and calcification of the falx cerebri. The study found that 80% of whites and 38% of African-Americans had at least one BCC, with the first tumor occurring at a mean age of 23 years for whites and 21 years for African-Americans. Jaw cysts were present in 74% of individuals, with the first tumor occurring in 80% by age 20 years. Palmar and plantar pits were observed in 87% and 81% of affected individuals, respectively. Ovarian fibromas were diagnosed in 17% of women, and medulloblastoma occurred in 4 patients. Physical findings included coarse face, relative macrocephaly, hypertelorism, frontal bossing, pectus deformity, and Spengel deformity. Radiological signs included calcification of the falx cerebri in 65%, tentorium cerebelli in 20%, bridged sella in 68%, bifid ribs in 26%, hemivertebrae in 15%, fusion of vertebral bodies in 10%, and flame-shaped lucencies in the hands and feet in 30%. The study discusses the diagnostic criteria for NBCC and provides recommendations for surveillance and management, emphasizing the importance of early detection and regular follow-up.