Background <p>Neurofibromatosis Type 1 (NF1) is a multisystem genetic disorder that may impact psychological well-being in children. This study aimed to assess behavioral problems and functional competence in children with NF1 and examine the associations of these problems with disease severity and lesion visibility.</p> Methods <p>A case-control study was conducted with 38 children diagnosed with NF1 and 38 ageand sex-matched healthy controls. All participants underwent an assessment using the Arabic version of the Child Behavior Checklist (CBCL). Clinical severity and lesion visibility were rated using the Modified Ablon Scale.</p> Results <p>Children with NF1 showed significantly higher scores in anxiety, attention problems, and social difficulties scales of the CBCL (<i>p</i> &lt; 0.001), along with greater scores on the internalizing and externalizing problem scales. Total competence scores, including school performance, social adaptation, and activities, were significantly lower in the NF1 group. Visibility and severity of physical lesions were positively correlated with behavioral problems and negatively correlated with competence. DSM-oriented scales revealed a higher prevalence of ADHD, affective symptoms, and PTSD-related complaints in the NF1 group.</p> Conclusion <p>These findings highlight the need for early psychological evaluation and multidisciplinary care strategies to support mental health and quality of life in this vulnerable group.</p> Impact <p><UnorderedList Mark="Bullet"> <ItemContent> <p>This study provides the first controlled data on emotional and behavioral problems in Egyptian children with NF1 using a validated Arabic CBCL tool.</p> </ItemContent> <ItemContent> <p>Children with NF1 showed more frequent internalizing, externalizing, and DSM-oriented psychiatric symptoms compared with healthy peers.</p> </ItemContent> <ItemContent> <p>Greater clinical visibility and severity of NF1 were associated with increased behavioral difficulties.</p> </ItemContent> <ItemContent> <p>Routine psychological screening and multidisciplinary management are mandated in children with NF1.</p> </ItemContent> </UnorderedList></p>