PURPOSE: The main purpose of this study was to describe the relationship of mothers' parenting attitude as perceived by the child to self-esteem, and school adjustment of school age children with Tetrology of Fallot (TOF). METHODS: In this study a self-administered questionnaire survey was used to collect the data. The participants included 38 children who were registered in a pediatric cardiology clinic in one tertiary medical center. Their ages were between 11 and 15 years. They were diagnosed with TOF, and had no other congenital problems. Data were collected from November 1 to November 30, 2009. After obtaining telephone consent from the mothers and children, questionnaires were mailed to 64 participants and 38 questionnaires were returned. Data were analyzed using Statistical Package for the Social Sciences (SPSS) WIN 15.0 version. RESULTS: There was a significant correlation between perceived parenting attitude, self-esteem, and school adjustment of children with TOF. CONCLUSION: Children who perceive their mothers' parenting attitude to be more positive also report higher self-esteem and better school adjustment. These findings are similar to other studies done with healthy school age children. However, parents of children with TOF may require different parenting approaches to foster positive self-esteem and school adjustment.