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Introduction Rhabdomyosarcoma (RMS) arises from mesenchymal cells committed to skeletal muscle. It is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. In this population, there are two main histological forms, which are the embryonal or the alveolar RMS. The retro auricular location is extremely rare. We present 2 cases of children with left retro-auricular RMS occurring at a very early stage of post natal development. Case reports Two children were included in the RMS 2005 Protocol. The first child, aged 5-days, was managed by surgical resection in two steps after a previous incomplete resection which was followed by 8 chemotherapy cycle. The second, aged 3-days, was managed by surgical resection of the tumor bed, completed by 8 chemotherapy cycle. On regular follow up for over 6 years after the diagnosis, both patients are free of recurrence. Discussion RMS is a fast-growing malignant and aggressive tumor originating from skeletal muscle. It occurs in the first decade of life and is associated with genetic conditions. With an unusual clinical presentation and anatomical location, both benign and malignant tumors can be suspected. Diagnosis can be performed with CT-scan or MRI and confirmed by biopsy. The treatment is based on chemotherapy followed by radiotherapy or surgical resection. In our two patients, the treatment program achieved complete disease control, with good prognosis especially because of anatomical location as well as early diagnosis.