Abstract
Extra-gastrointestinal stromal tumors (EGISTs) are rare mesenchymal tumors accounting for less than 1% of total gastrointestinal tumors. They tend to be aggressive and have a poor prognosis. Unfortunately, there is a lack of data or controversial data due to its scarcity. Therefore, we report a case of pelvic EGIST misdiagnosed as retroperitoneal sarcoma. We opted for surgical management followed by adjuvant oral chemotherapy with imatinib.