Dextrocardia with situs solitus and severe mitral regurgitation is a rare clinical presentation which posse a surgical challenge and requires specific preoperative planning. A 54-year-old women with this anatomy, multiple thoracic procedures, and severe mitral valve regurgitation underwent successful mitral valve replacement with a 27-mm mechanical prosthesis through a left thoracotomy under ventricular fibrillation, on the basis of computed tomography findings. We emphasize the importance of preoperative planning and a surgical approach through a left thoracotomy and under ventricular fibrillation.