Acinar cell carcinoma (ACC) of the pancreas is a rare tumor, and many aspects remain unclear because no large-scale clinical studies have been conducted. The present study investigated the clinical characteristics, treatment, and therapeutic outcomes of 115 patients registered in the Pancreatic Cancer Registry of the Japan Pancreas Society, and therapeutic plans were reviewed. Although ACC has been associated with advanced stage and poor prognosis, this tumor was resectable in 76.5% of the patients, and the 5-year survival rate after resection was favorable, being 43.9%. Confirming the diagnosis of ACC preoperatively is difficult, but this diagnosis should be kept in mind while planning surgery for ordinary pancreatic cancer. Once the diagnosis has been confirmed, a possibility of surgical resection should be pursued to achieve better prognosis. If ACC is unresectable or recurrent, chemotherapy is likely to prove useful. Multidisciplinary therapy centering on the role of surgery will need to be established.