Abstract Unicuspid aortic valve (UAV) is a rare congenital malformation, related to an aggressive evolution of valve function. Its diagnosis represents an important cardiac imaging challenge, since it is often difficult to differentiate between other anomalies like bicuspid aortic valve. Recently, bicuspid aortic valve has been associated with IE, especially when aortic valve function shows significant stenosis, however, the relationship between IE and UAV is unclear. The anatomical characteristics of UAV could predispose to IE. This report describes a young patient affected by infective endocarditis (IE) with UAV and the case reviews the echocardiographic criteria used to improve its management.