Spindle cell type anaplastic carcinoma of the pancreas is extremely rare and has a very poor prognosis. A previously healthy 68-year-old woman was referred to our hospital due to a large tumor in the body of the pancreas. Abdominal computed tomography revealed an irregular, well-enhanced 140-mm tumor containing a cystic component. The patient underwent distal pancreatectomy for a possible malignant tumor (e.g., mucinous cystic neoplasms). Histological examination showed that the tumor contained spindle-shaped cells and adenocarcinoma with nuclear atypia, and a definitive diagnosis of anaplastic carcinoma spindle cell type was made. A review of 27 cases reported in Japan revealed 43% of these lesions invaded other organs, and 26% were classified in Stage IV at the time of diagnosis. Postoperative recurrence rate was 78.2%, and mortality rate was 59.3%. Early diagnosis and sequential radial surgery would improve the poor prognosis.