Objective We aim to compare the perioperative blood loss and risk of allogeneic transfusion between hemophilia arthritis (HA)patients and osteonecrosis of the femoral head (ONFH) patients underwent unilateral total hip arthroplasty ( THA) under multimodal blood management measures. Methods: 26 patients with HA who underwent unilateral THA from January 1, 2010, to June 15, 2022, and met the criteria, with a mean age of (30.46±6.78) years and BMI of (23.90±4.53) kg/m2, were retrospectively included; 76 patients with ONFH who underwent unilateral THA were matched according to 1:3. All were male, mean age (34.41±7.398) years, BMI (24.61±4.11) kg/m2; demographic data, median operative time, median hospital stay, preoperative and postoperative day 1 and 3 hemoglobin and hematocrit, postoperative hemoglobin and hematocrit decline values, total blood loss, transfusion ratio, and complications were compared between the two groups. Results: There was significantly more total blood loss ((1927.08±956.60) mL vs. (1475.88±924.43) mL, P=0.036) and hidden blood loss (1526.70±835.82 mL vs. (1147.94±930.10) mL, P=0.069), higher rate of major bleeding (38.46% vs. 15.38%, P=0.002); higher transfusion risk (15.38% vs. 9.89%, P=0.012); longer median operative time (98min vs. 77min, P=0.015); and longer median hospital stay (20 days vs. 12 days, P=0.000) in HA-THA group than in ONFH-THA group. Conclusion: The risk of perioperative blood loss and allogeneic transfusion is still high in unilateral HA-THA compared to ONFH-THA patients. Further research is needed on prophylactic and effective methods to reduce the risk of blood loss in HA-THA.