Is presented the case a patient with Gorlin Goltz's syndrome, a disorder clinicopathological hereditary autosomal dominant well recognized and variable expression. In most cases is manifested sooner or around puberty affecting more Caucasian men and women. It is characterized by multiple basal cell nevus associated with other abnormalities. If a patient is 18 years old, white race that has multiple basal cell carcinomas on his face from childhood, which is associated with other anomalies such as winged scapula, bifid ribs and fused, syndactyly, vertebral anomalies, calcification of the falx brain, associated with mild mental retardation. Mother with similar picture. It is arrived at the diagnosis of Gorlin Goltz's syndrome. Conventional treatment was performed for carcinomas with favorable response. Following a year with good outcome. Very interesting case presentation by a flora of the warning signs and symptoms documented it.