Hepatobiliary and pancreatic ascariasis (HPA) was described as a clinical entity from Kashmir, India in 1985. HPAis caused by invasion and migration of nematode, Ascaris lumbricoides, in to the biliary tractand pancreatic duct. Patients present with biliary colic, cholangitis, cholecystitis, hepatic abscesses and acute pancreatitis. Ascarides traverse the ducts repeatedly, get trapped and die, leading to formation of hepatolithiasis. HPA is ubiquitous in endemic regions and in Kashmir, one such region, HPA is the etiological factor for 36.7%, 23%, 14.5% and 12.5% of all biliary diseases, acute pancreatitis, liver abscesses and biliarylithiasis respectively. Ultrasonography is an excellent diagnostic tool in visualizingworms in gut lumen and ductal system. The rational treatment for biliary ascariasis is to give appropriate treatment for clinical syndromes along witheffective anthelmintic therapy.Endotherapy in HPA is indicated if patients continue to have symptoms on medical therapy or when worms do not move out of ductal lumen by 3 weeks or die within the ducts. The wormscan be removed from the ductal systemin most of the patients and such patients get regression of symptoms of hepatobiliary and pancreatic disease.