Endomyocardial fibrosis is a common disease in tropical countries, particularly Uganda and Nigeria. It may affect the right and left ventricles, creating restrictive heart failure, which, in addition to the classical symptoms, manifests as ascites disproportional to peripheral edema. We report the case of a female patient with endomyocardial fibrosis refractory to clinical treatment, who underwent surgical treatment with clinical improvement for a short period. Three months after the surgery for resection of the endomyocardial fibrosis and tricuspid valvuloplasty, the disabling symptoms reappeared. Then the patient underwent bicaval orthotopic heart transplantation, which had a good clinical outcome. This was the first case of heart transplantation for the treatment of endomyocardial fibrosis, which proved to be a promising alternative.