ABSTRACT: Gorlin goltz syndrome, is a rare autosomal dominant inherited condition caused by the mutation in patched (PTCH),a tumor suppressor gene. It is clinically characterized with frontal and temporoparietal bossing results in the increased cranial circumference, multiple basal cell carcinomas, odontogenic kerotocysts, epidermal cysts of the skin, ocular telorism ,kyphoscoliosis, and various skeletal abnormalities. Here we present a case series of Gorlin goltz syndrome(two cases) in this article. This is an attempt to highlight its importance in early diagnosing this disease along with the review of literature.