Neurofibromatosis type 1 (NF1) is a genetic disorder associated with a high risk of the neuropsychological, emotional and psychosocial disturbances in children with NF1. In this research the results of the psychological and neuropsychological studies in children with NF1 were presented. According to data of current research, the most common cognitive deficits are connected with visual-spatial impairment, however verbal, working memory and executive function deficits associated with prefrontal cortex dysfunction are also important. The results indicate that the neuropsychological deficits may increase during the course of the illness.