Mesenteric fibromatosis (MF) is a rare, benign tumor commonly associated with Gardner’s syndrome. The signs and symptoms in patients with MF are insidious. Patients may present with abdominal pain or discomfort when the tumors reach large sizes, which is typical at the time of diagnosis. Differentiating MF from other neoplasms such as gastrointestinal stromal tumorsmay present a diagnostic dilemma, especially in patients without any history of familial adenomatous polyposis. In the present report, we discuss a young girl who presented with MF. A pertinent review of the literature is also presented. This case is peculiar in that MF presented at a young age in a patient without history of familial adenomatous polyposis and occurred in the omentum with local invasion to the stomach. Mesenteric fibromatosis (MF) is a rare, benign tumor normally associated with Gardner’s syndrome. The signs and symptoms in patients with MF are insidious. diagnosis. Differentiating MF from other neoplasms such as gastrointestinal stromal tumors can present a diagnostic dilemma, especially in patients without any history of familial adenomatous polyposis. In the present report, we discuss a young girl who presented with MF. A pertinent review of the literature is also presented. This case is peculiar in that MF presented at a young age in a patient without history of familial adenomatous polyposis and occurred in the omentum with local invasion to the stomach.