Pancreatic leiomyosarcomas are a rare neoplasm that accounts for 1/1000 of pancreatic cancers. In the literature, 23 cases of pancreatic leiomyosarcoma have been reported and the majority being diagnosed on autopsy. It has never been reported any radical curative surgery in presence of synchronous hepatic metastasis. We reported a case of a patient affected by a primitive pancreatic leiomyosarcoma with bilobar hepatic metastasis, who underwent distal splenopancreatectomy associated with the resection of multiple liver metastases.