Abstract
INTRODUCTION: Rhabdomyosarcoma is the most common malignant soft tissue tumor in the pediatric age, especially in the head and neck region; in the orbit and the nasopharynx. The middle ear is a very rare site for this neoplasm as it accounts for only 10 % of head neck rhabdomyosarcoma. PRESENTATION OF CASE: We report here the case of a three years-old child who was admitted to the emergency room for a left parotid and retro-auricular swelling with grade V facial palsy. The patient experienced chronic otorrhea with left facial palsy for two months. Two weeks later, a left parotid swelling appeared and gradually increased in size with weight loss. Computed tomography showed a slightly dense tissue lesion in the left mastoid. The patient underwent a diagnostic mastoidectomy. Pathology and immunohistochemical study were compatible with an embryonic rhabdomyosarcoma. CONCLUSION: Rhabdomyosarcoma should be considered as a differential diagnosis of any mastoiditis resistant to treatment in a young child. Because of its serious prognosis, rhabdomyosarcoma must be diagnosed at an early stage to increase chances of recovery.