Cardiac resynchronization therapy (CRT) is an acknowledged treatment for advanced heart failure in acquired dilated cardiomyopathy, resistant to pharmacotherapy. Although there are no therapeutic standards regarding heart failure originating from congenital heart defects with systemic right ventricle, a number of CRT implantations by transvenous approach in congenitally corrected transposition of the great arteries (CCTGA) have been reported since 2001, even though none of them expressly referred to a case concomitant with dextrocardia and situs inversus anomaly. We present a 57 year-old patient with dextrocardia and CCTGA, who underwent surgical closure of interatrial and interventricular septal defects at the age of 19, and in whom a VVI pacemaker was subsequently implanted at age 36. A three-lead CRT system was implanted by transvenous approach. Imaging techniques, including multi-slice computed tomography, targeted to pacing system and unusual anatomical relationships were applied. Within a 20-month follow-up, a significant improvement of functional NYHA class, systemic right ventricle ejection fraction and exercise capability were observed. Entirely transvenous CRT system implantation is feasible in patients with dextrocardia and CCTGA, and has substantial potential for long-term benefits.