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Endomyocardial fibrosis (EMF) is a tropical cardiomyopathy reported in many tropical countries. Patients were seen at the Children's Hospital-Khartoum, Sudan during September 2007–08 where cardiac evaluation was done. Six patients were identified (18% of all children with cardiomyopathy); all were males aged 8–17 years. All patients presented with abdominal distention, stunted growth, raised jugular venous pressure, ascites and hepatosplenomegaly. Electrocardiogram abnormalities included atrial fibrillation, first and third degree AV block and tall P wave. Echocardiography revealed huge right atrium dilatation and right ventricle apex obliteration by fibrous tissue. One patient had left-side affection with moderate mitral regurgitation. Eosinophilia was present in three patients. Two patients received antituberculosis drugs with no improvement, two had history of treated bilharziasis and two had been labeled as having Ebstein disease. EMF is an important cause of cardiomyopathy in Sudan that is often misdiagnosed. Clinical and echocardiographic examinations can lead to the correct diagnosis.