We report a case of alpha-fetoprotein (AFP)-producing acinar cell carcinoma (ACC) of the pancreas. The tumor was diagnosed in a 72 yearold female after radical subtotal gastrectomy (Billroth I) due to early gastric cancer six months before. The initial serum AFP levels were increased to 2,254.1 IU/ml and preoperative imaging studies showed a mass with approximately 2.5 cm in diameter near the neck of the pancreas. A pancreaticoduodenectomy was performed. The pathologic examination revealed an ill-defined lobulating tumor confined to the pancreas (T1 stage). Immunohistochemical study showed that the tumor cells expressed AFP. The Adenosine triphosphate-based chemotherapy response assay (ATP-CRA) suggested that cisplatin would be more desirable than gemcitabine in AFP-producing ACC of the pancreas as an adjuvant chemotherapy. However, the adjuvant chemotherapy was not performed due to the early pathological stage. The patient died from carcinomatosis and pneumonia. Even if the tumor was on a relatively early stage, an adjuvant treatment should be considered ACC.