AbstractBackgroundLeiomyosarcoma of visceral organs is uncommon, and pancreatic primary occurrence is even rarer. In terms of curative treatment, patients are generally managed with surgery alone, without significant data on the role or efficacy of adjuvant chemotherapy.Case presentationThis manuscript presents a case of a 22‐year‐old female with advanced primary leiomyosarcoma of the pancreas, treated with radical surgery and adjuvant radiation therapy.ConclusionWith a low‐survival rate, consideration of radiation therapy in some advanced and unresectable cases could be potentially beneficial.