Congenitally corrected transposition of the great arteries (L-TGA) is characterized by discordance between the atria and ventricles, as well as between the ventricles and the great arteries. As a result, the morphologic right ventricle lies to the left of the morphologic left ventricle and becomes the systemic ventricle. The morphologic right ventricle gives rise to the aorta, which is anterior of the pulmonary artery. As a result of discordance at both the atrioventricular (AV) and the great vessel level, physiologic blood flow remains normal. It was shown that the decreased RV functions are related with increased mortality rates in long term follow up. Most of these patients have RV failure due to abnormal pressure load of systemic RV, imbalance between O2 supply and myocardial demand and development of systemic AV valvular regurgitation in most of the patients. Therefore, the most important and difficult point in the follow up of these patients is evaluation of RV functions and degree of systemic AV valve regurgitation. In this review, we discussed the difficulties in the diagnosis and treatment of these patients in the light of a typical TGA case.