Objective:To discuss the clinical feature, diagnosis, and treatment course of pancreatic acinar cell carcinoma (ACC) to guide clinical practice and improve prognosis of patients. Methods:Clinical data of 15 patients with pathologically confirmed pancreatic acinar cell carcinoma between December 1994 and March 2014 in Tianjin Medical University Cancer Institute and Hospital were retro-spectively studied. Results:The patients include eight males and seven females with a median age of 44. Tumors in these patients appeared in different parts of the pancreas. Eight patients had tumor in the head, six in the body and tail, and one in the uncinate process. The tumor size ranged from 3 cm to 18 cm, with an average diameter of 6.67 cm. The patients presented less jaundice and the tumor markers remained constant, specifically, no increase was reported. Six patients had metastasis before their operation. Twelve patients received radical resection, while the other three received palliative treatment. The preoperative and intraoperative diagnoses were not exact. The final diagnosis depended on pathologic confirmation after surgery or puncture. The immunohistochemical results of trypsin and chymotrypsin were positive in the patients who were examined. The postoperative chemotherapy was usually based on gemcitabine. The average survival time was 20.6 months. Conclusion:Pancreatic acinar cell carcinoma has special clinical features, and clinicians tend to regard it as low-grade malignancy. The attitude towards ACC should be positive.