Objective: To investigate the clinical pathological features, and diagnosis and treatment as well as prognosis of pancreatic acinar cell carcinoma. Methods: The clinical data of 14 patients with pancreatic acinar cell carcinoma treated in the Department of General Surgery of China-Japan Friendship Hospital from January 1997 to January 2017 were retrospectively analyzed. Results: Of the 14 patients, 10 cases were males and 4 cases were females, with an average age of (51.6±15.6) years; the initial symptoms included abdominal pain in 7 cases, jaundice in 3 cases, nausea and vomiting in 1 case, and abdominal mass in 1 case, while 2 cases were asymptomatic and discovered by health examination. The average tumor diameter was (9.4±5.2) cm. The tumor was located in the body and tail of the pancreas in 10 cases (64%), in the head of the pancreas in 3 cases and the uncinate process in 1 case. All preoperative puncture biopsies and intraoperative frozen sections failed to identify pancreatic acinar cell carcinoma. R0 resection was achieved in 9 patients, and 5 patients received only pancreas biopsy or palliative resection due to liver metastasis or adjacent organ invasion. All patients were diagnosed as pancreatic acinar cell carcinoma by postoperative pathological findings. The results of immunohistochemical staining showed that positive expression rates of α-antitrypsin, α-antichymotrypsin and cytokeratin (AE I/AE3) were 100%. The mean survival time of the 9 patients with R0 resection was (32.0±25.6) months, while that of 5 patients without R0 resection was only (4.0±0.8) months. Conclusion: For pancreatic acinar cell carcinoma with a likelihood of R0 resection, aggressive surgical resection should be performed, even in the presence of liver metastases. Postoperative adjuvant chemotherapy is associated with better prognosis.