Mesenteric fibromatosis is a rare, locally invasive, non-metastasizing type of intra-abdominal fibromatoses with a very high rate of recurrence. Because of rarity, these tumors pose a diagnostic and therapeutic challenge. These tumors may remain asymptomatic, but commonly present as a painless mass. There are no clear treatment guidelines. Surgical excision with wide margin is the preferred modality of treatment. Medical therapy is indicated for inoperable and recurrent tumors. There are several isolated case reports on mesenteric fibromatosis with different and unusual presentations and its complications. Though several studies have been published on extra-abdominal fibromatosis as well as on extramesenteric abdominal fibromatosis, but extensive research studies are still lacking on mesenteric fibromatosis. The aim of this article is to present current knowledge on mesenteric fibromatosis, our experience of four cases, and comprehensive review of available literature.