Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, which primarily affects the skin in young children. Although intracranial involvement is rare, it differs from other systemic JXG because its responsiveness to non-surgical treatment is poor. We present the case of a 2-year-old boy with left abducens nerve palsy. Imaging studies revealed a mass in the left Meckel's cave. Systemic examination showed no other abnormalities including his skin. After partial removal, JXG was diagnosed based on immunohistochemical evaluation. He underwent a stereotactic radiosurgery. Then, the mass decreased, and his abducens nerve palsy improved. This is the seventh case with isolated intracranial JXG, and the first case to be treated with radiosurgery. We consider that stereotactic radiosurgery is an excellent choice for treatment of intracranial JXG.