BACKGROUND: Hemophilic pseudotumor (HP) is a rare complication in patients with 
hemophilia. The lesion most frequently occurs in the long bones, pelvis, small 
bones of the hands and feet, or rarely in the maxillofacial region. 
Postoperative changes in HP are seldom arrested, whereas angiogenesis 
characterized by disturbed wound healing in HP may cause vascular malformations.
CASE SUMMARY: We report the case of an 11-year-old boy who was affected by 
maxillary intraosseous venous malformation. Enucleation of an HP without factor 
replacement was performed initially on the right side of the maxilla 3 years 
ago. The patient was referred to us because of painless swelling in the same 
location. Factor replacement and subtotal maxillectomy were performed. 
Pathological examinations revealed intraosseous venous malformation.
CONCLUSION: This study is the first to document the development of intraosseous 
venous malformation after enucleation of an HP in the maxillofacial region. 
Angiogenesis characterized by disturbed wound healing in patients with 
hemophilia may be pivotal in the pathogenesis of this condition.