"Groove pancreatitis", a form of segmental pancreatitis affecting the head of the pancreas, is localized within the "groove" between pancreas head, duodenum, and common bile duct. Differentiation between groove pancreatitis and pancreatic head carcinoma is often difficult. We report a case of groove pancreatitis in which a hypoechoic mass between the duodenal wall and pancreas was clearly imaged, and narrowing of the second portion of the duodenum and bile duct stenosis were also found. The diagnosis was confirmed by surgery (pylorus-preserving pancreato duodenectomy). The patient was relieved from abdominal pain post operation. Up to the present, the patient has been good condition. We review the clinicopathologic and radiologic features of groove pancreatitis in the Japanese literature and discuss the possible role of Santorini's duct in its pathogenesis. We consider that impacted protein plugs in Santorini's duct are a pathogenic factor in the development of groove pancreatitis. Therefore, the findings of Santorini's duct on endoscopic retrograde pancreatography are very important in the diagnosis of groove pancreatitis. Groove pancreatitis presents various clinical features, such as biliary stenosis, duodenal stenosis, and pancreatic mass, and often masquerades as pancreatic head carcinoma. This condition should be kept in mind in the differential diagnosis of pancreatic head carcinoma.