Objective To reveal the character of tricuspid valve in hypoplastic right ventricular heart syndrome (HPRVHS) using 3-dimensional echocardiography(3DE). Methods Twenty eight patients with HPRVHS were included in the study, and they were divided into two groups according the degree of tricuspid regurgitation. Eighteen normal children were included as the control. Volume of right ventricle(VRV), area of tricuspid annulus(TVA),proportion of the leaflets and the distance from tips of leaflets to the nearest papillary muscle were measured with 3DE. These data was standardized by body surface area. Results The volume of right ventricle in HPRVHS was significantly less than that in normal group. The area of tricuspid annulus was significantly correlated with volume of right ventricle(r = 0. 90, P < 0. 0001). In the tricuspid regurgitation group, the proportion of area of anterior leaflet to area of tricuspid annulus was significantly higher than non-regurgitation group and normal group(P <0. 0001). Respectively,the proportion of the area of posterior leaflet to the area of tricuspid annulus in regurgitation group was significantly less than normal and non-regurgitation group. The distance from tips of leaflets was significantly shorter in HPRVHS than in normal group. Furthermore, the distance from the tip of septal leaflet to the nearest papillary muscle was shorter in regurgitation group than non-regurgitation group ( P = 0. 0023). Conclusions 3DE could be applied for evaluating the pathologic change of tricuspid valve in congenital heart disease. In HPRVHS, hypoplastic of posterior leaflet and shortness of the cords of septal leaflet could be the cause of tricuspid regurgitation.