Management of congenital mitral valve disease is challenging because of a wide morphologic spectrum, frequent associated lesions, and small patient size. We evaluated the results of a repair-oriented policy.All consecutive patients with congenital mitral valve disease who underwent surgery between 1996 and 2006 were studied retrospectively. Patients with atrioventricular canal, atrioventricular discordance, or ischemic regurgitation were excluded.During this period, 71 children (median age 2.9 years, range 3 days-20.8 years) underwent surgery. All but 1 underwent primary mitral valve repair. Twenty-two (30%) were younger than 12 months. Associated cardiac lesions were present in 45 children (63%) and were addressed concurrently in 35; previous cardiac procedures had been performed in 17 patients (24%). Mitral incompetence was predominant in 60 (85%) and stenosis in 11 (15%). During a median follow-up of 47.8 months (range 2-120 months), 14 patients underwent 17 mitral reinterventions: 14 repairs and 3 replacements. After 60 months, overall survival was 94% +/- 2.8%; freedoms from reoperation and prosthesis implantation were 76% +/- 5.6% and 94% +/- 3.6%, respectively. There were 4 deaths, and all survivors remain in New York Heart Association class I or II with moderate (6 patients) or less mitral dysfunction.Surgical repair of the congenital mitral valve can be successfully performed with low mortality, satisfactory valvular function at midterm follow-up, and acceptable reoperation rate while obviating risks associated with valvular prostheses. Suboptimal primary repair was significant predictor for reoperation but re-repair was often successful.