ObjectiveThis article highlights the clinical presentation and management issues of unilateral vestibular schwannomas in children. We demonstrate how the presentation differs from neurofibromatosis type 2 (NF2) and from adult unilateral vestibular schwannomas.Study DesignThis article is composed of a series of three cases and a literature review.SettingThe study was performed at a university hospital (tertiary referral center).PatientsThree children, aged 9, 11, and 13 years, with histologically confirmed vestibular schwannomas were studied. All children under 16 years of age in the world literature with unilateral vestibular schwannomas were reviewed.InterventionAnalysis of presentation and surgical management of these three children and those children reported in the literature.Main Outcome MeasurePattern of presentation relative to children with NF2 and people with adult unilateral vestibular schwannomas.ResultsTwo patients had multiple cranial nerve weakness and recurrence, and one patient had successful removal of the tumor with preservation of all functions of the cranial nerves, including the facial nerve.ConclusionVestibular schwannomas in children are very uncommon. It is likely that it is the first manifestation of NF2, but it may also be a variant of sporadic vestibular schwannomas. A presentation of three cases and a review of 36 other cases in the literature demonstrates how the presentation is different from adult sporadic vestibular schwannomas and NF2 because it lacks primary audiological symptoms. The study also provides evidence of non-NF2 vestibular schwannomas presenting in children and suggests that it is likely that these are a variant of unilateral sporadic vestibular schwannomas. The search for the features of NF2 in these cases remains mandatory.