Abstract: Objective　To investigate the clinical characteristics of neurofibromatosis type I (NF1) in children with hypertension. Methods　Clinical data of a pediatric patient with NF1 presented mainly with hypertension was retrospectively analyzed, and related literature was reviewed. Results　we report a case of uncontrolled persistent hypertension in a 4-yearold child with more than 30 café-au-lait spots. The enhanced MRI of the renal artery showed the right renal artery stenosis and aneurysm. Gene test found a de novo heterozygous c.3623delT mutation in NF1. At last, he was diagnosed with NF1, but he had no neurofibroma, Lisch nodules, axillary freckles, and other performances of neurofibromatosis. His blood pressure was well controlled after percutaneous transluminal renal angioplasty.  Conclusions　For children of persistent hypertension with skin lesions only, NF1 should be considered and blood pressure be monitored. Once the vascular lesions were found, intervention should be done timely.