Valvular epithelioid hemangioendotheliomas (EHE) are exceptional. To the authors' knowledge only four cases have been reported. Herein is described an EHE incidentally detected in the mitral valve of a 69-year-old woman with chronic rheumatic valvular heart disease. The 0.4 cm lesion was situated in the anterior leaflet and was discovered in the pathological study after mitral valve replacement. The patient was alive and well 6 months after operation. Review of the literature including the present report, revealed that the mean age was 60.2 years (range, 49-69 years). Symptomatic patients had clinical features of valvular disease or embolism. Each of the four cardiac valves can be affected. Mean tumor size was 2.4 cm (range, 0.4-8 cm). In 40% of cases the EHE was an incidental finding at autopsy or in a removed valve. In two cases the involved cusp was affected by chronic rheumatic disease. In conclusion, EHE is a rare lesion that can be an incidental finding and it should be considered in the differential diagnosis of valve tumors. Although EHE can present a histologically benign appearance, the correct pathological diagnosis is clinically important because the lesion can be potentially malignant. Regular follow up is suggested due to this potential.