OBJECTIVES: Literature is poor of data about the occlusion in children affected by neurofibromatosis type 1 (NF1). This case–control study investigated the occlusal traits in a group of children with NF1. SETTING AND SAMPLE POPULATION: A hundred and fifteen children with NF1 were enrolled; non-NF1 controls were sequentially selected among subjects referred to the Pediatric Dentistry Department. MATERIAL AND METHODS: All patients underwent a clinical dental examination and a panoramic radiography. The following orthodontic variables were considered: molar relationship, overjet, overbite, cross-bite, scissor bite, and crowding/spacing. RESULTS: Class III molar relationship resulted significantly (p = 0.01) more common in children with NF1 than in the control group as well as the unilateral posterior cross-bite (p = 0.0017). Forty-three children with NF1 (37.3%) showed radiographic abnormalities; in one case, a plexiform neurofibroma was detected. CONCLUSIONS: An early orthodontic evaluation might be planned in the management of children with NF1 to prevent or decrease the need for extensive orthodontic interventions.