Objective To analyze the midterm outcome of patients with congenital aortic stenosis undergoing percutaneous balloon aortic valvuloplasty (PBAV) by single echocardiographic guidance. Methods The clinical data of 12 patients with congenital aortic stenosis who underwent PBAV by single echocardiographic guidance at Fuwai Hospital from January 2016 to November 2017 were retrospectively analyzed. There were 7 males and 5 females with an average age of 18.27±15.30 years. The preoperative peak pressure gradient was 61.8–110.0 (80.30±24.50) mm Hg, and 50% of patients had aortic regurgitation. Results All patients successfully underwent PBAV. Aortic annulus diameter was 18.65±3.17 mm and balloon diameter was 17.62±3.77 mm, with balloon diameter to annulus diameter ratio of 0.92±0.07. The peak transaortic gradient was 16-51 (36.72±12.33) mm Hg immediately after procedure, which was significantly different from the preoperation (P=0.000). During the follow-up period, the peak transaortic gradient was 21-58 (37.06±13.52) mm Hg, and there was no significant difference between the follow-up and immediate postoperation (P=0.310). Immediately after procedure and during follow-up, 58% of patients had aortic regurgitation, which was not statistically different from the preoperation (P=0.682). Conclusion Systematic use of Doppler echocardiographic guidance for PBAV is feasible, and that it is associated with a high success rate and a very low complication rate.