This case report describes the surgical-orthodontic guided-eruption of a deeply impacted macrodontic maxillary central incisor in a 10-year-old patient with Alagille syndrome (ALGS). In the first stage, orthodontic treatment with fixed appliance on deciduous teeth allowed to create enough space for the eruption of the maxillary right central incisor. The second stage included closed surgical exposure and vertical traction. After impacted tooth erupted in the proper position, accessory periodontal treatment and dental reshaping procedures may be indicated to camouflage macrodontic incisor with the adjacent teeth. This is the first report that presents a patient with ALGS undergoing orthodontic and surgical treatment.