Ascaris lumbricoides is a common parasite and the most serious and dramatic presentation is hepatobiliary and pancreatic ascariasis (HPA). Therefore, this study was planned prospectively to elucidate the clinical presentation of HPA and evaluate the efficacy and safety of endoscopic intervention. In this study we documented 77 consecutive patients with HPA from January 2000 to November 2005. All the patients had endoscopically proven HPA. A total of 77 patients were included in the study. The age ranged from 6 to 80 years, with the third decade most commonly (28.6%) affected. Females were 6 times more likely to be affected than males. The commonest presentation was biliary colic (97.4%); other presentations were acute cholangitis (15.6%), obstructive jaundice (9.1%), acute pancreatitis (6.5%), choledocholithiasis (6.5%), acute cholecystitis (6.5%) and liver abscess (2.6%). In this report 51 (66.2%) had living, 10 (13%) had dead and 16 (20.8%) had both living and dead worms. Choledocholithiasis was associated only with dead worms. From one to 23 worms were found in the biliary tree. In 94.8% of cases we had to remove the worm by wide papillotomy followed by basket extraction. We did not experience any major complications during or following the procedures. Three patients had recurrent HPA during the course of follow-up (1 to 12 months). The majority of patients with HPA presented with biliary colic. This should be kept in mind in the management of an acute abdomen, especially in tropical countries. Endoscopic extraction is a safe and effective procedure for the treatment of HPA.