Groove pancreatitis, a rare form of chronic pancreatitis affecting the “groove” between the superior aspect of the pancreatic head, the duodenum, and the common bile duct, was first described by Becker in 1973 and has remained a diagnostic dilemma for radiologists, pathologists, and clinicians since its first description. Groove pancreatitis is an extraordinarily rare form of pancreatitis, and only a few descriptions of it exist in the radiology and pathology literature. Even in the most specialized centers, many radiologists remain unfamiliar with the entity. Unfortunately, even when the possibility of groove pancreatitis is prospectively considered on the basis of the imaging features, a definitive diagnosis can be extraordinarily difficult, and an inability to distinguish groove pancreatitis from a primary duodenal, ampullary, or pancreatic malignancy often ultimately leads to surgery. This review will focus on the underlying pathophysiology of groove pancreatitis, its typical clinical and biochemical manifestations, its radiologic appearance, the differential diagnosis for abnormalities in the pancreaticoduodenal groove, and the correlation between the radiologic and histopathologic features of the process.