Abstract. Desmoid tumor (DT) is a locally invasive form of fibromatosis, comprising only 0.03% of all tumors. DTs occur more frequently in patients with familial adenomatous polyposis and Gardner's syndrome, as intra-abdominal or anterior abdominal wall tumors, whereas sporadic DTs are more likely to be extra-abdominal (only 5% of sporadic DTs are intra-abdominal). There is also an association of DTs with prior trauma, surgery, estrogen exposure and childbirth. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) are used for preoperative diagnosis and for the planning of the surgery. Following surgery, CT and MRI are used to detect recurrence and to monitor the tumor's response to radiotherapy or medical therapy for unresectable or recurrent tumors. We herein report a rare case of a sporadic giant intra-abdominal DT in a 28-year-old female patient without any predisposing factors, and highlight the importance of including DT in the differential diagnosis of huge intra-abdominal masses.