To investigate the incidence, risk factors, clinical manifestations and prognosis of intracranial hemorrhage (ICH) in children with hemophilia A in a center of China, we conducted a retrospective analysis of 126 children with hemophilia A at our hospital in recent 4 years. Thirty-six children with hemophilia A (including 19 severe cases, and 17 moderate cases complicated with joint diseases) received low dose factor VIII (FVIII) prophylaxis, and none of them had ICH. However, 13 cases of hemophilia A not given prophylaxis were complicated with ICH (12 severe cases, and 1 moderate case) and demonstrated an incidence of 10.3% (13/126) in all patients, and 28.6% (12/42) in severe cases. Of the 13 cases, 9 severe ICH cases had a definite history of head injury, accounting for 69.2%. Headache was common in children >3 years, but somnolence, irritability, gaze or convulsions in children <3 years. The most common findings of cranial CT scan included intracranial hematoma (9/13), and less commonly observed were subependymal hemorrhage and intraventricular hemorrhage. After administration of FVIII, all patients survived. Hematoma of 6 cases was observed during CT reexamination after 1-3 months. During the follow-up period, only one case had slight activity limitation on one side of the limb, but steadily recovered. Besides the decreased concentration of FVIII, trauma is the most common risk factor of ICH in children with hemophilia A. The active treatment can improve the prognosis of ICH in children with hemophilia A.