FMD is a rare disorder of the bone and connective tissue consisting of a characteristic facies, prominent bony supraorbital ridges, progressive mixed hearing loss, and a series of musculo-skeletal alterations including limited joint motion and poorly developed musculature, especially of the hypothenar and interosseous muscles of the hands. Other clinical findings include micrognathia and abnormal dentition with retained deciduous teeth. Roetgenographic examination demonstrates hyperostosis of calvarium with a “Nazi helmet” configuration of the cranial vault, thick frontal ridges with absence of frontal sinuses and splayed metaphysis of the long bones. A heart murmur has been reported in a third of the cases, however, no further delineation of the cardiovascular findings have been made.We would like to report a 15 year old patient with typical features of this disorder who had MVP diagnosed by abnormal physical and echocardiographic findings. With mounting evidence that MVP may be a systemic connective tissue disorder, the findings of MVP in our patient is most likely related to the underlying connective tissue defect. Although MVP tends to be a benign disorder, it has been associated with infective endocarditis, cardiac arrhythmias and sudden death. Antibiotic prophylaxis against infective endocarditis is mandatory. In light of the potential morbidity, it is recommended that patients with FMD be investigated for the presence of MVP.