Desmoid tumors are clinical entities rarely diagnosed at an initial presentation because of its low incidence, they are characterized by a locally aggressive presentation and high rates of local relapse. Its presentation can be intra-or extra-abdominal. We present a clinical case of a female, a 15-year-old patient, with 3 months of abdominal pain, a giant intra-abdominal mass was diagnosed with histologic diagnosis of desmoid tumor. Several surgical procedures were performed, having a R1 resection (focally microscopic margins). In this case, association with pregnancy, abdominal trauma, previous surgeries, and genetic syndromes were discarded.