Submitral aneurysm is an extremely rare cardiac anomaly which was initially described among the African population and is considered rare in other parts of the world. In majority of the reported cases aetiology is congenital. We report a unique case of 54-year-old immunocompetent female with submitral aneurysm and tuberculous endocarditis of the mitral valve. She underwent successful submitral aneurysmoraphy with mitral valve replacement. Postoperative recovery has been uneventful without relapse for 12 months.