Background: Unicommissural aortic valve (UAV) is diagnosed by means of echocardiography during systole, because absence of cusp separation reveals eccentric “teardrop” opening. Case Report: A 4-month infant was refereed for evaluation of murmur at aortic area by his pediatrician. On examination, peripheral pulses were weak with diminished and delayed carotid upstroke. Transrthoracic echocardiography revealed unicuspid, unicommissural aortic valve (UAV) with moderate aortic stenosis. There was a supra-aortic membrane without any aortic leak. Conclusion: The importance of its early diagnosis lies in its risk of sudden cardiac death which can be mitigated and other associated congenital anomalies can also be corrected.