Kartagener's syndrome is a rare autosomal recessive disease featuring the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Situs inversus is associated with dextrocardia wherein the cardiac apex lies over to the right. Acquired valvular lesions in this condition are rare and surgery on them is even rarer. The standard of treatment in a dextrocardiac mitral valve disease is a mitral valve replacement via a right atriotomy. Here, we present a case of mitral valve stenosis with Kartagener's syndrome, in whom a mitral valve replacement was performed via a left atriotomy.