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Abstract Background The management of mitral valve disease in young children is challenging. Mechanical mitral valves could provide long-term durability; however, the need for anticoagulation increases the risk profile of mechanical valves. We report our experience in mechanical mitral valve replacement (MVR) in children under 2 years of age and evaluate factors affecting the outcomes. The study included all patients younger than 2 years who underwent mechanical MVR between 2000 and 2023. The study outcomes were mitral valve reoperation, bleeding, valve-related thrombosis, and survival. Results Twenty-three patients were included, with a mean age of 10.2 ± 5.9 months. The mitral valve sizes ranged from 16 to 25 mm, and 6 (26%) were placed in the supra-annular position. Complete heart block occurred in seven patients (30%), and operative mortality occurred in three patients (13%). Postoperative warfarin was used in 17 patients (74%). After discharge, bleeding occurred in five patients (22%), four were managed conservatively, and one had intracranial hemorrhage treated with craniotomy. Nine patients (39%) had valve-related thrombosis; two underwent reoperation, while seven were treated with alteplase in 26 patients. Valve-related thrombosis was more common in patients with supra-annular valves (p