Endomyocardial fibrosis (EMF) is a neglected disorder, which is predominant in tropical regions and characterized by fibrotic thickening of the endomyocardium of one or both ventricles, resulting in a restrictive cardiomyopathy (1). Although eosinophilia, infections, toxic agents, malnutrition and immunologic causes have been shown to be associated with EMF, the etiology remains controversial (2). We present a rare case of right ventricular EMF with atrial septal defect (ASD).