Mitral valve repair is a better therapeutic alternative than valve replacement for rheumatic valve disease in children. Repair procedures are especially well suited to developing countries where heart prostheses and life-long anti-coagulation therapy are largely unaffordable. The purpose of this study was to evaluate medium-term outcome of mitral valve repair in children in Senegal. A retrospective review was conducted in a cohort of 100 patients who underwent mitral valve repair for rheumatic mitral lesions over the 8-year period from 1999 to 2007. Mean age was 12 +/- 5 years (range, 7 to 17 years). The most common symptom of valve disease was dysypnea (stage IV in 26 cases and stage III in 74). Valve lesions were complex with anterior leaflet prolapse in 62 cases, posterior leaflet restriction in 35, commissural fusion in 30, and fusion of chordaes in 54. Repair procedures consisted of transfer and shortening of chordaes in 73 cases in association with commissurotomy in 22 cases and cleft closure in 17. Ring annuloplasty was performed in 84 patients. Hospital mortality was 2%. Postoperative morbidity was characterized by residual mitral regurgitation in four cases. Mean follow-up was 5 years. No late deaths were observed. Outcome was satisfactory in 84 patients with low-grade mitral regurgitation (grade I-II). Reduction of left ventricle diameter was statistically significant during systole and diastole, i.e., from 29.5 +/- 6.2 mm to 33.1 +/- 5.3 mm (p<0.05) and from 47.1 +/- 8.6 mm to 50.5 +/- 9.4 mm (p<0.05) respectively. Improvement in cardiac function was not significant, i.e., from 63.3 +/- 4.8% to 62 +/- 6.4% (p = 0.99). Mitral valve repair was successful in stabilizing myocardial function and remodeling the left ventricle. Outcome is dependent on careful patient selection and evaluation of lesions. Middle-term outcome is encouraging.