Endomyocardial fibrosis (EMF) is a rare disease in North America but common in the tropical and subtropical regions of the developing world. It is characterized by fibrosis of the left ventricular and right ventricular endocardium which cause restrictive cardiomyopathy. In endemic areas of Africa, endomyocardial fibrosis is an important cause of heart failure accounting for up to 20% of the cases. Currently, the exact etiology and pathogenesis of the disease remain unknown. However, its pathology resembles conditions such as eosinophilic cardiomyopathy and hypereosinophilic syndrome. As a result, EMF is sometimes considered part of a single disease process that also includes Loffler endocarditis (eosinophilic endomyocardial fibrosis). It has been recognized that EMF is similar to the fibrotic stage of Loffler endocarditis, as severe chronic eosinophilia has been known to cause endomyocardial damage and fibrosis. Even though there is a resemblance between the two conditions, eosinophilia is not always seen in patients with EMF. Due to the geographical location of the disease, several infectious pathogens have been considered; these include Plasmodium species, Microfilaria, Schistosoma, Helminths, arboviruses, and Toxoplasma. Nonetheless, there has been no correlation with a single organism in EMF. Endomyocardial fibrosis is most prevalent in the tropical regions of Africa, Asia, and South America. It is a disease that mainly affects young adults of lower socioeconomic status. A study in Uganda indicated a bimodal peak at 10 and 30 years of age. Studies on gender distribution have been mixed, and currently, there is no specific data on gender predisposition.