Frontometaphyseal dysplasia is an extremely rare craniotubular bone disorder predominantly manifested by supraorbital bossing. Although recognizable with findings at birth, it is usually identified successfully before the onset of puberty. These patients often are stigmatized related to their appearance and may present to the plastic surgeon for intervention. We present a case of successful cranioplasty in correcting the fronto-orbital deformity in a 9-year-old child with frontometaphyseal dysplasia.