Anterior open bite (AOB) is often seen in patients with amelogenesis imperfecta (AI). The skeletal and dental components were analysed in 15 patients with AI and AOB. Measurements on cephalometric radiographs and dental models were compared with those of 130 patients with AOB but without enamel anomalies. Skeletal components in the AI and non-AI group were comparable. The AI group showed omega-shaped dental arches and a reversed mandibular curve of Spee. Orthodontic treatment options are limited because of the conical form of the teeth, tight contacts in the posterior regions and the condition of enamel resulting in difficulties in bonding brackets. Fixation problems encountered during surgery are discussed. A multidisciplinary treatment is outlined consisting of a multi-segment Le Fort I osteotomy followed by prosthetic rehabilitation to stabilize occlusion for at least one year postoperatively.