A 47-year-old male with history of alcohol abuse for 8 to 10 years visited the Department of Gastrointestinal Surgery for recurrent attacks of mild acute pancreatitis since 3 years. He also had recurrent epigastric pain, significant weight loss, early satiety and postprandial fullness. Multiple ultrasonography examinations and Computed Tomography (CT) scans of abdomen done in the past suggested chronic pancreatitis for which patient was advised pancreatic enzyme supplementation, alcohol abstinence, analgesics and dietary changes by different physicians, only to recur again.CT scan revealed bulky head and uncinate process of pancreas with a hypodense area between pancreatic head and second part of duodenum [Table/ Fig-1], prominent pancreatic duct, dilated Common Bile Duct (CBD) (15mm), hugely distended gall bladder, irregular wall thickening of first and second part of duodenum with stenosis of second part of duodenum [Table/ Fig-2]. Upper gastrointestinal endoscopy did not reveal significant duodenal obstruction. Duodenal biopsy showed Brunner's gland hyperplasia. Endoscopic Ultrasound (EUS) confirmed the CT findings. An Fine Needle Aspiration Cytology (FNAC) showed only inflammatory infiltrate and no malignancy. Blood level of CA19-9 was normal. Liver function tests were normal.A diagnosis of groove pancreatitis was made. In view of inability to exclude a neoplasm with certainty, the patient was subjected to a pylorus preserving Pancreatoduodenectomy (PD). During surgery a hard mass in the head and the uncinate process of pancreas was felt. There was no pancreatic calcification. A dilated CBD and a fibrotic partially constricting ring in the midportion of second part of the duodenum were noted.Histopathological examination [Table/ Fig-3] revealed islands of heterotopic pancreatic tissue in the muscularis propria of the duodenum. The lobules comprised of unremarkable pancreatic acini, ducts and few clusters of islet cells. The acini were atrophic in places and few aggregates of ductules were seen. Cystic dilatation of ductules was seen in the duodenal wall with mild to moderate inflammatory infiltrate. Extensive brunner gland hyperplasia was seen in mucosa and submucosa of the duodenum leading to widening of submucosa. The duodenal muscle was markedly hypertrophied, especially in its medial wall, which contained the heterotopic pancreatic tissue. Both these features probably led to narrowing of the duodenal lumen. The head of pancreas showed focal evidence of chronic pancreatitis. The postoperative course was uneventful. The patient is asymptomatic five years after the surgery. A diagnosis of groove pancreatitis was made. The patient was subjected to a pylorus preserving PD. Histopathological examination showed cystic dystrophy of duodenum due to heterotopic pancreas. The patient is doing well at 5 years of follow-up.[