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The orbital rhabdomyosarcoma is one of the most frequent malignant orbital tumours in children. At this age, the common histological types are the embryonal and alveolar type. The onset is mainly under the age of 16. Without a recent and correct treatment it can give metastasis in lung and bone. The hereby paper presents one clinical case of a teenager presented at the ophthalmological consultation for a small tumor located in the superomedial part of the orbit. Computed tomography (CT) and magnetic resonance imaging (MRI) supported the diagnosis revealing the location and extension of the tumor. During the surgery, we discovered two small tumors and the histological examination revealed an embryonal type of orbital rhabdomyosarcoma. After the surgery, the patient followed an oncological treatment consisting of chemotherapy and local radiotherapy. The prognosis for life was favorable, linked with the recent diagnosis and treatment, the histological type and the good response at the oncological treatment.
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