Abstract: 　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.