Primary pancreatic lymphoma (PPL) is a rare form of extranodal lymphoma (less than 0.5% of pancreatic tumors) originating from the pancreatic parenchyma. Histopathological examination is usually mandatory to obtain a definitive diagnosis since symptoms and radiological features are quite similar to those of other pancreatic masses. Percutaneous fine-needle aspiration (FNA) of the pancreas requires experienced cytopathologists as well as advanced immunohistochemical assays to obtain a final diagnosis on a small amount of tissue. A 46-year-old man complaining of watery diarrhea and severe weight loss (more than 20 kg) for more than 1 year was admitted to our hospital due to severe diabetic crisis. Enlarged lymph nodes (2.5 x 1 cm) were found at the right axillary stations. Abdominal ultrasound revealed the presence of a large hyperechogenic mass, mainly located at the pancreatic head. Abdominal computed tomography scan confirmed a diffuse enlargement of the head and body of the pancreas associated with lymphadenopathy along the lesser gastric curvature. Percutaneous ultrasound-guided FNA of the pancreas as well as gross biopsy of the axillary lymph nodes were unable to identify the nature of the mass. Diagnostic laparoscopy was performed: several enlarged lymph nodes along the lesser gastric curvature were revealed. Multiple biopsies of the pancreatic head were taken and lymphadenectomy along the lesser curvature and the hepatic hilus was also performed. The definitive histopathological examination of the pancreatic specimen revealed a primary low-grade non-Hodgkin B cell pancreatic lymphoma. The postoperative course was unremarkable; the patient underwent systemic chemotherapy regime for low-grade B cell Hodgkin lymphoma and he was symptom free at 9-month follow-up.