Objectives This study aimed to evaluate the safety and efficiency of percutaneous transluminal angioplasty (PTA) with selective stenting treating Chinese patients with renal artery stenosis (RAS) due to fibromuscular dysplasia (FMD). Background Endovascular data of patients with RAS caused by FMD are scarce in China. Methods Clinical data of 105 hypertensive patients with RAS caused by FMD underwent endovascular therapy at a single-site between June 2001 and October 2018 were analyzed retrospectively. Baseline blood pressure, use of antihypertensive medications, renal function, and adverse events at a 1-year follow-up were evaluated. Results The patients were aged between 10 and 64 years (mean age 26.7 +/- 8.2 years) and 52 (49.5%) were female. In total, 105 patients undergoing endovascular therapy involving 124 RASs. Thirty (24.2%), sixty-four (51.6%), and thirty (24.2%) RASs were multifocal, unifocal, and tubular types, respectively. Ninety-four (89.5%) and eleven (10.5%) patients underwent PTA alone and PTA plus stenting, respectively. The technical success rate for endovascular therapy was 95.2% (100/105) without severe complications. During 1-year follow-up (n = 100), the mean systolic and diastolic blood pressure decreased from 157.6 +/- 17.5 and 102.3 +/- 14.2 to 129.6 +/- 12.3 and 81.3 +/- 11.1 mmHg, respectively, and the number of antihypertensive medications reduced from 2.2 +/- 1.2 to 0.8 +/- 1.0 (all p < .001). The cure rate and improved rate of blood pressure was 49.0 and 40.0%, respectively. The serum creatinine levels remained stable. The primary and secondary restenosis rate was 13.4 and 5.8%, respectively. Conclusion Endovascular therapy for treating RAS caused by FMD was technically safe and effective for reducing blood pressure in Chinese patients.