A 42-year-old male was referred to our hospital for epigastric and back pain. Following a diagnosis of acute pancreatitis, abdominal CT revealed a low-density tumor measuring 15 mm in diameter in the pancreatic body. Endoscopic retrograde pancreatography showed narrowing of the main pancreatic duct, and cytology of pancreatic juice indicated class Ⅲ. Given the strong suspicion of pancreatic cancer, a distal pancreatectomy was performed. Pathological examination confirmed spindle cell-type anaplastic carcinoma of the pancreas, categorized as pT2, pN0, M0, and pStage ⅠB. Local recurrence and liver metastasis were observed on postoperative day 72. The tumor progressed rapidly, leading to the patient's death on postoperative day 115. Anaplastic carcinoma of the pancreas has a poor prognosis, emphasizing the urgent need for establishing multidisciplinary treatment.