Anatomical variations of pancreatic head and uncinate process are rarely encountered in clinical practice. These variations are primarily attributed to the complex development of the pancreas. An unduly enlarged uncinate process of the pancreas overlapping the third part of duodenum was discovered during dissection. This malformation of the pancreatic uncinate process was considered to be due to excessive fusion between the ventral and dorsal buds during embryonic development. On further dissection, an avascular pancreatico-duodenal fold guarding the pancreatico-duodenal recess was observed. The enlarged uncinate process can cause compression of neurovascular structures and also cause compression of adjoining viscera. The pancreatico-duodenal recess becomes a potential site for internal herniation. This case is of particular interest to the gastroenterologists and surgeons performing surgical resections. Precise knowledge of embryogenesis of such pancreatic anomalies is necessary for understanding and thus treating many diseases of the pancreas.