Background Neurofibromatosis type 1 (NF1) is a genetic disorder associated with neurocutaneus manifestations, as well as attention and learning problems. The aim of this study was to examine the psychosocial functioning, quality of life, and self‐image of children with NF1. Method Two hundred forty participants were recruited, comprising 60 children and adolescents with NF1, 60 parents of children with NF1, 60 children and adolescents without NF1, and 60 parents of children without NF1. The children/adolescents completed the Self‐administered Psychiatric Scales test for Children and Adolescents and the human figure drawing test, whereas parents completed the Child Behavior Checklist. Both children and parents completed the Pediatric Quality of Life Inventory. Results Compared to healthy subjects, individuals with NF1 reported anxiety problems, poorer quality of life, and greater distortions in terms of self‐image. When compared to healthy participants' parents, the parents of NF1 participants reported having more concerns about the quality of life, sociality, school performance, and attention span of their children. Conclusions Results show that the psychological functioning, behavior, self‐image, and quality of life of children and adolescents with NF1 are compromised. Health services should take into account the psychosocial difficulties associated with NF1 and design rehabilitation programs aimed at increasing NF1 children's interpersonal skills, improving their social life and quality of life, and promoting more adaptive behaviors. In addition, health care interventions should also involve the parents of children with NF1.