PURPOSE: Acinar cell carcinomas (ACCs) are a rare pancreatic tumor group with no standardized treatment. The aim of the study is to analyze the clinical and pathologic characteristics of our series and to review the current literature. METHODS: Retrospective review of prospectively collected data from 1990 to 2007 included patients who underwent pancreatic resection for histologically proven ACCs. All specimens of ACC were rereviewed by an expert pathologist. Follow-up was updated to October 2009. A literature search was performed by Pubmed and COCHRANE library. RESULTS: Among 1,210 patients who underwent pancreatic resection, we identified nine ACCs. R0 resection was possible for all but one R1. We had no major complications and no mortality. All nine cases were diagnosed as pure ACCs. Five patients received adjuvant chemotherapy. Median overall survival was 31 months, while median disease-free survival was 18 months. All patients developed liver metastases, requiring modification of chemotherapeutic schema, radiofrequency ablation techniques, or reiterate surgery. Currently, only one patient is alive without evidence of disease 85 months after pancreatic resection. One patient is alive 52 months after operation, with evidence of recurrent disease. CONCLUSIONS: ACC represents a rare solid tumor of the pancreas. Prognosis is dismal, although, compared to the more common ductal adenocarcinoma, survival appears to be longer. Patients with metastatic disease might benefit from aggressive multimodality treatments.