Uhl anomaly is a rare form of congenital hypoplasia of the right ventricular 
myocardium. Here, we report, a rare finding in fetal cardiac ultrasound in a 
33-year-old woman who presented at 20 weeks' of gestation. A diagnosis of Uhl 
anomaly was made. An autopsy was performed at 23weeks gestation after obtaining 
permission for medicolegal termination of pregnancy. Histopathological 
examination confirmed the diagnosis. Diagnosing Uhl anomaly in fetal life is 
essential since mortality and survival mainly depend on the severity of right 
ventricle dysfunction related to, the either partial or complete absence of the 
myocardium. Hence, surviving cases need to be followed up carefully and 
counselled accordingly.