Total situs inversus (TSI) with dextrocardia is an infrequent anatomical characteristic and anterior papillary muscle rupture (PMR) is one of the rare mechanical complications following myocardial infarction. A 56-year-old man who had a history of mitral valve regurgitation (MR) presented with dyspnea to the emergency department. Upon transesophageal echocardiography, there were dextrocardia and chordal flail in the A1 scallop. Coronary angiography showed a substantial stenosis of the left anterior descending artery (LAD). Computed tomography of the thorax and abdomen showed TSI. During the operation, dextrocardia and chordal flail with partial anterior PMR at the A1 segment were observed. Following the mitral valve repair (MVr) with an artificial neo-chord and the ring annuloplasty implantation, coronary revascularization (LAD-right mammarian artery) was performed. On the control TOE, he had no MR. Conclusion: Patients who had TSI with dextrocardia and partial anterior PMR can undergo successful MVr.