A 48-year-old man presented with chronic right sided heart failure. 2D echocardiography revealed the classical features of left ventricular endomyocardial fibrosis with a prominent right ventricular apical aneurysm. Right ventriculography further defined the aneurysm. Cardiac magnetic resonance images revealed a thin-walled, apical aneurysm of right ventricle with multiple septations and marked obliteration of left ventricular apex. A delayed-enhancement sequence after the infusion of contrast, demonstrated a hypersignal in the subendocardium, consistent with the right ventricular involvement of endomyocardial fibrosis. This patient had classical features of left ventricular endomyocardial fibrosis, while on the right side the typical features were missing. This aneurysm may be a passing phase of the natural history of endomyocardial fibrosis before the development of burned out stage. This aneurysm may later develop thrombus, and which may progress to fibrosis and apical obliteration. Endomyocardial fibrosis with right ventricular aneurysm has not heretofore been reported in the medical literature.