血友病是由于机体缺乏凝血因子而导致的一种遗传性出血性疾病，主要为凝血因子Ⅷ（factor Ⅷ，FⅧ）缺乏的血友病A及凝血因子Ⅸ（factor Ⅸ，FⅨ）缺乏的血友病B。随着凝血因子替代治疗的应用，国内外关于血友病患儿手术治疗的报道逐渐增多［1-4］。神经外科手术本身术中及术后出血风险较高，若合并严重出血性疾病，围手术期风险明显增加。近年来国外已间断有血友病合并颅内恶性肿瘤手术的报道［5］。北京大学第一医院小儿外科于2013年9月至2014年3月收治2例颅内恶性肿瘤合并血友病A患儿，均行开颅手术治疗，围手术期平稳，术后顺利出院，现结合文献分析如下。

Hemophilia A is aninherited bleeding disorder, lack of coagulation factor Ⅷ (FⅧ), and if combined with intracranial malignant tumor, the operation risk is very high. Department of Pediatric Surgery in Peking University First Hospital used coagulation factor replacement therapy, succeeded in the operation of 2 cases of intracranial malignant tumor with hemophilia A in children, with no abnormal bleeding events. The establishment of the multi subject cooperation group before operation, good preoperative preparation, enough alternative factors, and close postoperative monitoring, are the key to the successful treatment.