Pseudotumor is an uncommon but severe complication in patients with hemophilia. To our knowledge, although China has large population of persons with hemophilia, there is rare information on the incidence, clinical feature, image finding, and management of pseudotumor among Chinese patients. This study aimed at improving our knowledge on clinical diagnosis and management of hemophiliac pseudotumor. In this retrospective study, the medical records of 1248 patients with hemophilia diagnosed between January 1983 and October 2004 at our hospital were reviewed. The clinical feature, imaging finding, management, and outcome of 14 patients with pseudotumor among these patients with hemophilia were analyzed. All patients have hemophilia A (8 severe cases and 6 moderate cases). Eight patients sustained an injury prior to the development of pseudotumor. Main image findings included osteolysis lesion, soft tissue swelling, or lump. Surgical therapy was carried out in 7 patients and 6 achieved remission, with fistula formation remaining in 1. One patient underwent radiotherapy together with replacement therapy achieved remission. Three patients accepted replacement therapy as only management and only 1 patient achieved improvement of swelling. Our study showed that the incidence of pseudotumor in our enrolled patients with hemophilia is 1.12%. Hemophilic history of patients can contribute to the right diagnosis of pseudotumor. Surgical therapy together with sufficient replacement therapy is safe and effective.