Primary pancreatic lymphoma (PPL) is a rare disease that is difficult to diagnose preoperatively. We describe the youngest case of PPL treated by surgical excision and chemotherapy. A 16-year-old male presented with abdominal pain and jaundice. Abdominal computed tomography showed a 3.0 × 4.5 cm homogeneously enhanced mass localized between the inferior vena cava and pancreatic head; the common pancreatic duct was dilated and the common bile duct was stenosed. Magnetic resonance imaging findings showed a 4.5 cm tumor localized between the inferior vena cava and pancreatic head with low signal intensity on T1W images and high intensity on T2W images, which enhanced inhomogeneously. Endoscopic retrograde cholangiopancreatography findings were compatible with smooth stenosis of the common bile duct. He was diagnosed as pancreatitis secondary to pancreatic tumor and pylorus-preserving pancreaticoduodenectomy was performed. Postoperative diagnosis was PPL and chemotherapy was performed. After 4 years of treatment he has no signs of recurrence.