Based on a review of the current literature, only 29 cases of primary pancreatic leiomyosarcomas have been reported [26]. These tumors can range from 1.0 to 30.0 cm in diameter, with an equal distribution among men and women. Symptoms are usually present at the time of diagnosis; although none of these are specific, patients may complain of abdominal pain, jaundice, and weight loss. Aggressive surgical treatment is usually advocated, as these neoplasms respond poorly to systemic treatments. Pancreatic leiomyosarcomas tend to metastasize to other organs, especially the liver whereas lymph node metastases are unusual.