Abstract
INTRODUCTION: Primary hepatolithiasis is a rare disease in western countries and it is associated with repeated attacks of acute cholangitis. Without proper treatment, hepatolithiasis can lead to progressive biliary strictures and secondary biliary cirrhosis. PRESENTATION OF CASE: A 40 years old male was admitted due to recurrent cholangitis during the last 18 years. Bilateral primary hepatolithiasis was diagnosed and treated by left hepatectomy with an intrahepatic hepaticocutaneous jejunostomy. There were no postoperative complications and the patient was discharged after 7 days. DISCUSSION: The management of patients with primary hepatolithiasis remains a challenging task due to the high incidence of residual and recurrent stones after treatment. CONCLUSION: Primary bilateral hepatolithiasis is a complex disease that can be managed with partial hepatectomy with an intrahepatic hepaticocutaneous jejunostomy.