Objective: To evaluate the clinical efficacy of modified ultrasound-guided percutaneous transhepatic catheterization plus cholangioscopic lithotomy in treatment of hepatolithiasis complicated with acute obstructive cholangitis. Methods: The clinical data of 10 patients with hepatolithiasis and acute obstructive cholangitis undergoing ultrasound-directed percutaneous transhepatic catheterization and cholangioscopic lithotomy from June 2013 to June 2013 were retrospectively analyzed. Results: In the 10 patients, ultrasound-guided puncture and catheterization was successfully performed; the pain was relieved 2-3 h after tube placement, while temperature returned to normal level and symptoms of pain and chills disappeared 24-48 h after tube placement. Tube retention time ranged from 40 d to 2 months with an average of 50 d. The 10 patients successfully underwent cholangioscopic stone removal and dilation of bile duct stricture via sinus tract after 6- to 8-week tube placement, and the stone-free rate was 100%. Dark red-colored bloody bile was continuously drained from the drainage tube in one patient 10 min after the puncture and catheterization, which was controlled by intraluminal injection of reptilase and catheter occlusion. No serious complications such as bile leakage or surrounding organ injury occurred in any of the 10 patients. Conclusion: Modified ultrasound-guided percutaneous transhepatic catheterization plus cholangioscopic lithotomy is reliable and effective method for hepatolithiasis with acute obstructive cholangitis, and is recommended to be used.