The use of balloon aortic valvuloplasty for congenital aortic valve stenosis was well established in literatures. However, balloon aortic valvuloplasty performed in low body weight neonates had been infrequently reported. Here we report a 5-day-old premature neonate diagnosed critical aortic valve stenosis. Balloon aortic valvuloplasty was performed as first-line therapy while the patient weighed only 1493 g. Balloon aortic valvuloplasty went successfully with transvalvular pressure gradient decreased from 80 mmHg to 44 mmHg. Aortic regurgitation after balloon aortic valvuloplasty was mild. The patient's clinical condition stabilized after balloon aortic valvuloplasty and was able to gain weight to 2665 g. Our report demonstrates that balloon aortic valvuloplasty is possible, safe and efficient as first-line approach for critical aortic valve stenosis in neonates with low body weight.