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Cystic Dystrophy and Heterotopic Pancreas (CDHP) is a rare cause of chronic pancreatitis, duodenal obstruction and obstructive jaundice. The diagnosis is often delayed and requires multimodal imaging with Computed Tomography (CT), Magnetic Resonance Cholangio-Pancreatography (MRCP) and Endoscopic Ultrasound (EUS). The treatment is not standardized but Pancreatoduodenectomy (PD) provides long-term relief in some cases. The present case is about a 47-year-old male with history of alcohol abuse for 10 years who presented with a history of recurrent acute pancreatitis. CT scan revealed bulky head of pancreas with a hypodense area between pancreatic head and second part of duodenum, prominent pancreatic duct, Dilated Common Bile Duct (CBD) (15mm) and stenosis of second part of duodenum. EUS confirmed CT findings. A Fine Needle Aspiration Cytology (FNAC) showed only inflammatory infiltrate. 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.