Shortage of donor has pushed the transplantation centers to change the selection criteria of donors especially for pediatric recipients. Clinical studies in ABO incompatible heart transplantation are among the most important advances in this regard. All the reported cases of ABO incompatible heart transplantation are infants and young children and as far as we know this is the first report of a 14 year old patient undergoing this procedure. The paper presents the first ABO incompatible heart transplantation in Iran with 18 month follow up. The perfusion strategy and induction therapy are described. The literature review of published articles relating this topic is provided. All the cares and drug regimens used for this case was as routine. She experienced two rejection episodes treated with corticosteroid in the first and Anti-Thymocyte Globulin (ATG) in the second episode. She is now doing well and the Echo findings are excellent (EF=55%). The review of literature provided stresses the differences between our experience and the reported cases. The surprising therapeutic effect of ATG is discussed as well. ABO incompatible heart transplantation proved to be quite safe in infants and young children may be safely used in selected non-pediatric cases. The immunosuppressive regimen in these cases should not necessarily be changed but rejection surveillance and treatment needs special considerations.