Uhl's anomaly was first reported by Uhl in 1952 and is characterized by 
congenital partial or complete absence of right ventricular myocardium. It is a 
very rare anomaly with unknown aetiology. Associations with other congenital 
heart diseases, familial occurrency, sudden death and arrhythmia with Uhl's 
anomaly have been reported. Pathologic findings vary with the patient's age and 
severity of the right ventricular disorder. In infancy, it may occur with severe 
right-sided heart failure as well as asymptomatic cardiomegaly. Despite its 
rarity, Uhl's anomaly may be considered in patients with right ventricular 
failure due to dilated cardiomyopathy of the right ventricle. We report the case 
of six-year-old boy presenting with striking ascites due to severe right heart 
failure of Uhl's anomaly.