Gorlin-Goltz syndrome (GGS) is an infrequent autosomal do-minant multisystemic disease with complete penetrance and variable expressivity. It is estimated to have an incidence of 1:50,000 - 1:150,000 cases with a M/F = 1:1. This report describes a case of recurrent abdominal pain due to a large mesenteric cyst in a 38-year-old female patient affected by a rare disease: Gorlin-Goltz syndrome.