Sclerosing mesenteritis is a rare benign disorder of unknown etiology that often resembles a neoplasm. Numerous terms have been used in the past to describe this entity which has a variable clinical presentation and is characterized by thickening of the intestinal mesentery. Accurate information regarding the epidemiology, natural course and therapeutic response of the disease is sparse. Herein we present a review of the literature and a brief discussion of relevant concepts for the diagnosis and treatment of patients with sclerosing mesenteritis.