It was probably Jarisch in 1894 who first described a few characteristics of this syndrome in one of his patients with multiple basal cell epitheliomas, who also had a high degree of scoliosis, and low intelligence. Although Straith in 1939 commented on the association of mandibular cysts with basal cell epitheliomas, the suggestion of associated developmental or congenital defects was not correlated until Binkley and Johnson reported a patient in 1951 with basal cell naevi, dental cysts, agenesis of corpus callosum, a bifid rib and a fibroma of the ovary. In 1960 the association of multiple basal cell epitheliomas, jaw cysts, and bifid ribs was recognised by Gorlin and Goltz as a syndrome. A number of additional features of this syndrome have since been described. The purpose of this paper is to review multiple characteristics of this syndrome and present two cases in a family. This syndrome principally consists of multiple basal cell naevi/carcinomas, maxillary and mandibular epithelial lined cysts, ske...