The rhabdomiosarcoma is the third most frequent extracraneal solid tumor in childhood and is the most frequent sarcoma in this age group. 1/3 of these comprise the head and neck area, and only 4% are present in the middle ear as its primary site. The Fundacion Hospital de La Misericordia (HOMI) is a 4th level hospital committed with the attention of pediatric population and it is a national referral center for the management of high complexity pathologies. We will make a revision of ear rhabdomiosarcoma at our institution and a review of the literature to socialize symptoms and pronostic factors in Colombia.