Stenosis of the mitral valve most often occurs as a result of chronic rheumatic heart disease, causing thickening and fibrosis of the mitral valve apparatus. Rheumatic heart disease continues to be a major public health problem in several developing countries and mitral stenosis is also common in these regions. According to the reports from India and Africa, the disease tends to follow a rapidly progressive course in children. The treatment of choice is balloon dilatation of the mitral valve. Echocardiography is indispensable for this procedure. Before planning the procedure, it is essential to assess the suitability of balloon dilatation. Echocardiography performed during the procedure helps to decide whether the size of the balloon needs to be increased in case of inadequate relief of stenosis. Most published series have reported an immediate success rate of over 90% with balloon dilatation in children and young adults. With an increase in mitral valve area and improvement in functional class, the left atrial pressure and the transmitral gradients fall. These gratifying results are also reported from very young children of less than 12 years of age. It is recommended to start with a smaller balloon size and increase its size in a stepwise fashion to minimize complications. The complications, seen in about 1% to 2% of cases, include development of significant mitral regurgitation and hemopericardium, secondary to cardiac chamber perforation. The long-term results indicate slightly higher restenosis rates in children than in adults. Most children with restenosis can undergo successful repeat dilatation.