Objective To analyze clinical presentation, diagnosis, treatment options, and outcome of pancreatic schwannoma. Methods A retrospective study of clinical data of a case in our hospital and 71 cases reported in literature with pancreatic schwannoma. Results 72 cases were analysed. The mean age was 54 years (range 17-89 years), with 56 % of patients being female. Mean tumor size was 6.1 cm (range 1-20 cm). Tumor location was the head (29 cases), body and tail (32 cases), and uncinate process (6 cases). 27 cases exhibited solid tumors and 28 cases exhibited cystic tumors. Treatment included pancreaticoduodenectomy (23 cases), distal pancreatectomy (15 cases), enucleation (16 cases). 5 cases (7%) were malignant schwannoma. Tumor size of malignant group was significant larger than benign group (13.8±6.2 cm vs 5.6±4.1 cm,P=0.0004). There was no local recurrence metastasis,or death at the follow-up after operation (range 3-65 months). Conclusion The clinical manifestations of pancreatic schwannoma are lack of specificity and preoperative diagnosis remains difficulty. The tumor size was significantly related to classification of malignant or benign. Pancreatic schwannoma has satisfactory prognosis with surgical treatment.