Objective -Uhl's anomaly, a very rare anomaly with unknown aetiology, is characterized by congenital hypoplasia of the right ventricular myocardium. In this paper, we aim to discuss the clinical findings and the imagining methods. Case report -A six-month-old case was referred us for cardiomegaly. Echocardiography showed a hugely dilated and diffuse hypokinetic right ventricle, magnetic resonance images and cardiac catheterization proved the diagnosis of Uhl's anomaly. Conclusion -Uhl's anomaly

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... is a rare cause of cardiomegaly, and echocardiography and cardiac MR images are beneficial in the diagnostic process of this anomaly.