Male patient 47 years old who started his condition with epigastric pain and palpable mass. Imaging studies and biopsy of the pancreas were performed; biopsy was reported as primary sarcoma of the pancreas; metastatic lung radiographically and local extension to duodenum; with the aforementioned data a primary sarcoma of the pancreas clinical stage IV was diagnosed. The patient underwent surgery and subsequently received chemotherapy. It reached an overall survival of 48 months, the highest reported in the literature so far.