Neurofibromatosis type 1 (NF1) is a genetic condition that affects different systems, including the nervous system. Children with NF1 are at risk of experiencing various cognitive and behavioral challenges. An overlap with symptoms associated with other neurodevelopmental disorders, including attention deficit hyperactivity disorder (ADHD) and autism spectrum disorder (ASD), is also present in this genetic condition. . Studies carried out with animal models of NF1 have identified imbalances in neurotransmission which could underlie cognitive deficits. How these imbalances translate into brain activity in humans with NF1 remains little understood to this day. The general objective of this thesis is therefore to study, using electroencephalography (EEG), the neuronal responses during sensory processing in children with NF1 as well as the associations with cognitive and behavioral functioning. The results of the thesis made it possible to identify particularities in the electrophysiological responses of sensory processing in children with NF1 and to put forward measures whose translational potential could facilitate the transfer of knowledge accumulated in animal models of NF1.