A woman, aged 30 years with situs inversus, dextrocardia and severe rheumatic mitral restenosis was referred to AFIC/NIHD for commissurotomy. A Closed Mitral Valvotomy (CMV) for severe mitral stenosis had already been done 8 years earlier, and her symptoms had reappeared. She was evaluated with transthoracic and transesophageal echocardiography and found to have a mitral valve area of 0.6 cm2, dextrocardia along with atrioventricular (AV) and ventriculo-atricular (VA) concordance was demonstrated. Successful percutaneous transvenous mitral commissurotomy (PTMC) was done. Transseptal catheterization was done via the left femoral vein. Pigtail catheter was placed in the noncoronary aortic sinus; interatrial septal puncture was done with the transseptal needle rotated to a 7 O'clock position. There were no procedural complications. Reduction in trans-mitral pressure gradient on cardiac catheterization data, and standard echocardiographic parameters confirmed a successful procedure. PTMC can be accomplished safely in patients with this unusual cardiac anatomy with a few modifications in the standard technique, even if surgical treatment has already been carried out.