Although fibromatosis of the mesentery is a very rare locally aggressive benign condition, the uncertain treatment modalities, the natural history of the disease, and the other common differential diagnosis of the condition along with inexperience of the general clinicians with this disease pose a challenge to the professionals. The prolonged periods of stability and even regression in size of the tumor offer a hope for treatment. Accounting for 0.03% of all neoplasms, it is also known as deep fibromatosis and desmoid tumor. Here, we discuss one case of primary mesenteric fibromatosis in a young male patient who presented to us with chronic abdominal pain after he was treated for acid peptic disease for the same at a local hospital. This case shows how management of this disease can be delayed due to unfamiliarity among clinicians of this condition. In our patient, a palliative surgical management plan was undertaken due to symptomatic mass in the abdomen, owing to unresectability.
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