A case of congenital solitary tricuspid regurgitation was reported. The patient was a 43-year-old male. The echocardiogram and ventriculography showed severe tricuspid regurgitation. The tricuspid valve was hypoplastic in each leaflet and chordae without torn chordae and papillary muscles. A tricuspid valve replacement was performed using St. Jude Medical 33 mm valve. As of June 1993 nine patients with this disease undergoing surgery were reported around the world.