Objective To investigate the effectiveness and safety of ERCP in treatment of hemophilia A patients. Methods The data of 7 cases with hemophilia A including 5 cases mild type and 2 cases moderate type in our hospital performed ERCP from December 2013 to March 2017 were retrospectively analyzed. Five cases had choledocholithiasis including 1 case combined with biliary infection and other 2 cases with biliary obstruction of pancreatic head cancer. Anti-hemophilic globulin (AHG) was given perioperatively combined with accurate monitor of coagulation factor level as well as vital signs. Results There were 2 cases with endoscopic papillary balloon dilation and bile duct stone removal, 2 cases with endosco-pic sphincterotomy and bile duct stone removal, 2 cases with endoscopic metal biliary drainage and 1 case with endosco-pic nasobiliary drainage (ENBD) only. Totally, ENBD was performed for 4 cases of bile duct stone. All patients discharged with hospital stay (9.6± 3.2) (7-16) d. The preoperative FⅧ:C was (18.7%± 11.1%) (1.2%-28.2%) and AHG dose was (2 743± 964) (2 000~4 000) U/d. There was no significant difference in the postoperative hemoglobin (100± 30)(51~131) g/L and hematocrit (0.293± 0.084) (0.160-0.396) when compared with those preoperatively (105± 35) (50-150) g/L and (0.307± 0.097) (0.161-0.423). The postoperative serum amylase was (105± 80)(28-237) U/L. No any ERCP perioperative complications were found including bleeding, gastronintestinal perforation and acute pancreatitis. Conclusions ERCP could be effective and safe for patients with hemophilia A after infusion of AHG and careful monitoring and perioperative treatment.