INTRODUCTION: Gorlin syndrome is a rare autosomal dominant disorder characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence is 1/57,0001 to 1/256,0002. Main clinical manifestations include multiple basal cell carcinomas, odontogenic keratocysts, skeletal abnormalities, ovarian fibromas and other disorders. CASE: A 48 year old woman presented with multiple brown-black to skin-colored pearly macules, papules and nodules over the scalp, face, neck, trunk, upper and lower extremities. Histological examination at 2 sites revealed basal cell carcinoma. This was accompanied by findings of odontogenic keratocysts, palmar pits, posterior falx calcification, exotropia and multiple myoma uteri. Electrodessication with curettage of superficial basal cell carcinomas (< 1 cm.) was combined with wide excision of nodular basal cell carcinomas on the left temporal area (followed by rotational scalp flap reconstruction), right lateral breast, left inframammary area, right and left anterior thigh. CONCLUSION Gorlin syndrome is a hereditary condition affecting various organ systems. Management requires a multidisciplinary approach and regular medical surveillance is required.