目的　分析原发性膀胱毛细血管扩张症的诊断思路。方法　回顾1例原发性膀胱毛细血管扩张症患儿的临 床资料。结果　患儿，女， 9岁。近3岁时无明显诱因出现肉眼血尿，以后多次呼吸道感染后诱发的间断肉眼血尿，血凝块， 持续性镜下血尿，曾伴失血性休克1次。尿常规蛋白+ ~ ++，红细胞满视野；24 h尿蛋白定量0.96 g；腹部、泌尿系超声、 泌尿系增强CT扫描未见异常；肾动脉造影未见动静脉畸形或瘘；膀胱镜检查提示毛细血管扩张症。患儿无相关家族史， 无其他部位毛细血管扩张。遗传性毛细血管扩张症和共济失调-毛细血管扩张症相关基因突变分析未见异常。结论　儿 童原发性膀胱毛细血管扩张症临床较为罕见，对于表现为早发、长期、间断肉眼血尿伴血凝块，特别有失血性休克者，应 首先考虑血管因素病变可能，常规行泌尿系统各种影像学，包括血管造影检查，必要时膀胱镜检查。

　Objective　To explore the diagnosis of primary bladder telangiectasia. Methods　The clinical data of a child with primary bladder telangiectasia were reviewed. Results　A 9-year-old girl had gross hematuria without obvious cause at 3 years old. After that she presented intermittent gross hematuria and persistent microscopic hematuria with blood clots in the urine following repeatedly respiratory tract infections, and had hemorrhagic shock once. Urine routine examination showed albumin 1+~2+ and RBC full in entire field of view. 24 hours urine protein quantitation was 0.96 g. Ultrasound of abdomen and urinary tract and enhanced CT of urinary system had no abnormal findings. Renal artery angiography showed no arteriovenous malformation or fistula. Cystoscopy showed telangiectasia. There was neither family history nor telangiectasia in other parts. Both genetic telangiectasia and ataxia telangiectasia gene mutation analysis were normal. Conclusion　It is rarely seen primary bladder telangiectasia in children. However, children with early onset, long-term, and intermittent gross hematuria with blood clots, especially suffered with hemorrhagic shock, vascular disease should firstly be considered. And routine urinary imaging should be performed, including angiography and ,if necessary, cystoscopy.