Diagnosis and treatment of Caroli disease associated with hepatolithiasis WANG Jian, YANG Lin-hua. Department of General Surgery, Renji Hospital ，Medical College of Shanghai Jiaotong University，Shanghai 200127，China Corresponding author: WANG Jian, E-mail：dr_wangjian@yahoo.com.cn Abstract The causes of intrahepatic stone formation are possibly related to the chronic biliary inflammation and cholestasis. The non-invasive image exams such as B ultrasound, CT and MRI are useful to clarify disease type and ascertain the lesion area. Treatment principle of the disease should follow that of the hepatolithisais. For type Ia patients, lobar or segmental hepatectomy should be used. For type Ib patients, cystojejunostomy with large diameter in low position should be used after stones are eradicated. For type II patients, of whom the lesion is diffuse, liver transplantation is the most effective method. But for those with cyst dilation combined with stones in lobar and local bile ducts, who cannot accept liver transplantation, biliojejunostomy or jejunum-interposition-biliodudenostomy should be done after the plastic operations of hilar biliary tracts and stone eradication. When cholangitis occurred, Radical operations such as liver lobectomy or regional resection should be carried out after outward drainage such as PTCD had been used to control the acute inflammation.