Groove pancreatitis is a rare form of segmental chronic pancreatitis which is localized within the head of the pancreas, the duodenum and the common bile duct. Symptoms are due to common bile duct stenosis or duodenal stenosis. Radiologically, there is a pancreatic mass, which hinders differential diagnosis with pancreatic carcinoma. We report here a case of groove pancreatitis observed in a 41-year-old man treated by pancreatoduodenectomy. Histological features of the groove scar were noted. Our case and cases reported in the literature lead to hypotheses concerning the pathogenesis and clinical, biological, and radiological features suggestive of the diagnosis.