AIM: We investigated the clinical presentation and management issues of unilateral vestibular schwannomas in childhood without evidence of neurofibromatosis. MATERIAL and METHODS: Ten pediatric patients with unilateral vestibular schwannoma and no evidence of neurofibromatosis were treated by the senior author at Tiantan Hospital from January 1994 to December 2014. The clinical manifestations, neuroimaging findings, treatment methods, and therapeutic results were retrospectively reviewed. RESULTS: The study included 6 male and 4 female patients. The mean age of the patients was 13.9 years (range, 11-15 years). Common clinical features included increased intracranial pressure, hearing loss, and ataxia. The median diameter of the tumors was 5.15 cm. Gross total resection was achieved in 9 of 10 patients via a suboccipital retrosigmoid approach, and the perioperative mortality rate was 10%. The follow-up period ranged from 1 to 12 years. One patient with facial nerve injury recovered to House-Brackmann grade I postoperatively, 5 to grade II-III and 2 to grade IV-V. Only 1 patient had preserved hearing. CONCLUSION: The clinical features of pediatric vestibular schwannomas are different from those of adult vestibular schwannomas, and cranial nerve preservation in children is more difficult. When a child presents with hearing loss as well as cognitive disability, a vestibular schwannoma should be highly suspected. Hypervascular vestibular schwannomas in childhood should be managed by multistaged surgeries.