Ascarias lumbricoides infestation is endemic in tropical countries. Most infections are asymptomatic, but it can produce a wide spectrum of manifestations including hepatobiliary and pancreatic complications. There are reports of association of biliary ascariasis with bilary malignancies in the past, but same is not known for pancreatic ascariasis. We report a case of association of periampullary malignancy with pancreatic ascariasis.