Abstract
Rhabdomyosarcoma (RMS) is the most common soft-tissue malignancy in children under 15 years of age. Approximately, 35% of RMS cases originate from the head and neck region. Among various sites in the head and neck region, intracranial extension is more likely to occur with parameningeal tumors, which is also considered an unfavorable prognostic factor in children with RMS. About 20% of RMS occurs in a parameningeal site. Intracranial RMS are rare tumors that usually arise from parameningeal sites or metastasis from an extracranial site. Primary intracranial types are even rarer, with only 50 reported cases of primary intracranial RMS in the literature. Hereby, we report the case of a 6-year-old boy who presented with clinical and radiologic features of a cerebellopontine angle lesion, which turned out to be a RMS.