Temporal bone rhabdomyosarcoma mimicking otitis media complicated by facial nerve palsy

Background Rhabdomyosarcoma is common in childhood, especially, the head and neck region, yet involvement of the temporal bone is rare. Case presentation We reported a case of an embryonal rhabdomyosarcoma in a 4.5-year-old boy presenting with external auditory canal polyp and purulent otorrhea that later developed grade 6 facial palsy. Imaging showed soft tissue mass involving the middle ear, mastoid cavity, parotid gland, and parapharyngeal space. Subtotal petrosectomy with blind closure of the external auditory canal was performed with facial nerve decompression and debulking biopsy followed by combined chemoradiation. Conclusion Middle ear rhabdomyosarcoma is a rare pathology, usually present in childhood by symptoms similar to suppurative otitis media not responding to medical treatment leading to delayed diagnosis and development of complications.