In this report, we describe the case of a 67-year-old woman with metastatic pancreatic uterine leiomyosarcoma. She underwent a total hysterectomy and adnexectomy in December 2009. The resected uterine specimen was characterized as a leiomyosarcoma. The patient was free of disease until November 2010, when three pulmonary tumoral lesions detected by follow-up chest computed tomography were diagnosed as metastatic lesions. Wedge resections and enucleoresection of the pulmonary tumoral nodules were performed, and the patient received adjuvant chemotherapy. Ten months after the lung resection, an abdominal examination showed two tumoral masses in the pancreas and no extrapancreatic recurrence. In April 2014, a pylorus-preserving pancreaticoduodenectomy was performed. To date, the patient is alive, without any evidence of recurrence, and she has received chemotherapy. Surgery can be considered in cases in which the pancreas is a unique metastatic site or even in cases with resectable oligometastases.