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Desmoid tumors (DTs) are rare locally aggressive benign soft tissue tumors with an estimated annual incidence of two to four new cases per million people. The giant intra-abdominal mass presents a diagnostic challenge that includes a broad differential diagnosis of gastrointestinal stromal tumor (GIST), fibrosarcoma, retroperitoneal fibrosis, and other malignancies from adjacent organs. We report a case of a 38-year-old male patient with a giant intra-abdominal mass. Magnetic resonance imaging (MRI) of the abdomen and pelvis indicated mucinous cystic neoplasm or gastrointestinal stromal tumor (GIST), but histopathology confirmed it to be a desmoid tumor. The patient was discharged, and on follow-up five months until now, there is no recurrence. This case highlighted the importance of including DT in the differential diagnosis of very large intra-abdominal masses.

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