Endomyocardial fibrosis (EMF) is a neglected tropical cardiomyopathy of unknown etiology and pathogenesis that is common in certain tropical areas of Africa, Asia, and South America. It affects predominantly children and young adults. Endocardial fibrosis is the hallmark of this restrictive cardiomyopathy leading to restriction to diastolic filling of the ventricles with severe atrial dilatation. Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients. The salient features of this condition are present in the case presented here of a 14-year-old boy who died from progressive heart failure due to right ventricular EMF. These pathological findings of advanced disease highlight the need for early diagnosis and better understanding of the pathogenesis in order to improve prognosis of this debilitating and fatal disease.