Invasion of the Ascaris worm into the biliary system leads to a wide variety of clinical syndromes. Most of the descriptions of the disease have originated from the developing world, where due to the environmental factors there is a high level of parasitism. An increased incidence of biliary ascariasis borne out of population migration and increased facilities for diagnosis has led to a renewal of interest in this disease in the developed world. Significant morbidity and mortality is associated with the concomitant complications, and early diagnosis and management is of utmost importance. Common disease presentations include biliary colic, obstructive jaundice, acalculous cholecystitis, choledocholithiasis, pancreatitis, and cholangitis. Although with a potential for serious mortality, pancreatitis, and cholangiocarcinoma constitute relatively less common threats. Recent advances in endoscopy have shifted the attention of this disease from the surgeon to the gastroenterologist and a consensus of opinion is arising for early intervention. We present here a patient with biliary ascariasis managed endoscopically and review the epidemiology, prevalence, clinical presentation, diagnosis, and management of this disease.