Intraductal Papillary Mucinous Neoplasm (IPMN) of the pancreas in association with Autosomal Dominant Polycystic Kidney Disease (ADPKD) is extremely rare, even though 10% of ADPKD patients may develop simple pancreatic cyst. The first case report was published by Yasunori Sato from Japan in 2009. Since then less than 10 case reports are available worldwide to describe about this condition. We reported such a rare case of a 67-year-old man with ADPKD who was referred to our centre because of chronic abdominal pain and diagnosed as IPMN based on the serial imaging procedures. Despite of the high risk comorbidities, he successfully underwent pylorus preserving total pancreaticoduodenectomy with splenectomy.