Acinar cell carcinoma (ACC) of the pancreas is a rare tumor of the exocrine pancreas accounting for about 1% of pancreatic neoplasms. ACC is usually a well-circumscribed solid neoplasm. In most cases ACC is first diagnosed during histological examination of the resected specimen. Characteristic clinical symptoms of ACC have not been described. Patients usually show symptoms related to the effects of a local mass and/or the presence of metastases. Some patients present with symptoms related to lipase hypersecretion syndrome. Surgical resection seems to be the only curative treatment option and the same oncological operations as for ductal adenocarcinoma of the pancreas (PDAC) should be used, such as pancreatoduodenectomy and distal pancreatic resection. The prognosis of patients with ACC is essentially determined by the possibility of surgical resection and seems to be better than for PDAC. The benefit of adjuvant therapy remains unproven. Chemotherapy alone should be used for patients with nonresectable locally advanced or metastatic disease.