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Non-Hodgkin lymphoma predominantly involving the pancreas is a rare tumor and accounts for less than 0.7% of all pancreatic malignancies and 1% of extranodal lymphomas. Diagnosis of primary pancreatic lymphoma can be difficult because it may mimic carcinoma. The principal aims of this review were to highlight the difficulties encountered in making a diagnosis and to identify the role of surgery.A PubMed search was conducted using the following terms: primary pancreatic lymphoma and non-Hodgkin lymphoma of the pancreas. Additional references were sourced from key articles.A total of 89 reported cases of pancreatic lymphoma between 1951 and 2005 were reviewed. An accurate preoperative diagnosis of primary pancreatic lymphoma is not always possible. A complete response rate of 100% and a long-term survival rate of 94% have been reported with surgery and adjuvant chemotherapy when compared with a 5-year survival rate of less than 50% and an overall 3-year disease-free survival rate of 44% with current chemotherapy, radiotherapy, or combined methods.Pancreaticoduodenectomy may have a therapeutic role in association with chemotherapy.