BackgroundHaemophilia and von Willebrand disease (VWD) are common inherited bleeding disorders. Although patients with haemophilia or VWD have a high risk of hepatitis virus infection and hepatocellular carcinoma (HCC), little is known about the safety of liver resection in these patients.MethodsFrom 2006 to 2016, there were seven hepatectomies with haemophilia A and three hepatectomies with VWD for malignant liver tumours at tertiary care hospitals in Japan and Switzerland. To evaluate the safety of hepatectomy in the blood coagulation disorder group (BD group), short‐term outcomes in these patients were compared with 20 hepatectomies (non‐BD group) for HCC, matched to a 2:1, operative procedure, period and background liver.ResultsTen liver resections were performed in patients with haemophilia or VWD with administration of recombinant FVIII or VWF concentrate. Comparison of the BD vs non‐BD group revealed no significant differences in the operative time (327 vs 407 minutes, P = 0.359), estimated blood loss (730 vs 820 mL, P = 0.748), red blood cell transfusion rate (10.0% vs 5.0%, P = 0.605), major complication rate (Clavien‐Dindo grade III or IV) (10.0% vs 5.0%, P = 0.605) or mortality rate (0% vs 0%, P > 0.999). Additionally, the length of the postoperative hospital stay was similar between the two groups (13 vs 14 days, P = 0.296).ConclusionLiver resection for treatment of HCC in patients with haemophilia or VWD can be safely performed through an appropriate perioperative administration protocol of coagulation factors.