Primary pancreatic lymphoma (PPL) is a rare entity, most likely to be clinically misdiagnosed as pancreatic cancer. The cure rate of PPL is higher compared with that of pancreatic adenocarcinoma. This is the case report of a 57-year-old male patient who was hospitalized with complaints of abdominal pain, weight loss and jaundice. The radiological evaluation revealed a pancreatic head mass and, following endoscopic ultrasound-guided fine-needle aspiration biopsy, the tumor was diagnosed as diffuse large B-cell lymphoma. The final diagnosis was PPL, and the patient went into remission after receiving three cycles of treatment with rituximab, doxorubicin, cyclophosphamide, vincristine and prednisolone (R-CHOP regimen). Therefore, PPL should be considered in the differential diagnosis of pancreatic masses and its management differs from that of other types of pancreatic tumor.
