Until the Japanese Organ Transplantation Act was revised on 17 July 2010, only persons who had a written consent for organ donation after brain death could donate their organs in Japan, and children under 15 years of age could not donate their hearts after brain death. Therefore small children could not undergo a heart transplantation (HTx) in Japan, and many Japanese children traveled abroad to undergo HTx. After revision of the Act, small children were able to donate organs if their family agreed; ultimately, six children (one younger than 6 years, three from 10 to 14 years, and two from 15 to 17 years) donated their hearts through 2014. In this review, the current status and issues of pediatric HTx in Japan and indication of pediatric HTx and management before and after HTx are described. Briefly, most indications of HTx were dilated cardiomyopathy (DCM) and restrictive cardiomyopathy in Japan, and in Japan many candidates with DCM required a left ventricular assist device (LVAS) for bridge to HTx. The immunosuppressive regimen was calcineurin inhibitor and mycophenolate mofetil, and steroid was discontinued within 6 months in most children. Patient survival at 10 years after HTX was 100% in children transplanted in Japan and 87.6% in children transplanted abroad. Posttransplant lymphoproliferative disorder and various infections were major morbidity and mortality. Non adherence should be paid attention, especially to take care of adolescent patients.