Rhabdomyosarcoma is a soft tissue highly malignant neoplasm. The most common sites in childhood are the head and neck with only 10% of all cases occurring in the orbit. Clinical manifestations are not specific which makes pre-operative diagnosis challenging. Orbital tumors are mostly detected at early stages, due to their obvious symptoms but they are characterized with a rapid growth. Imaging techniques and especially MRI are very contributive in the diagnosis process. The purpose of this review is to provide a general overview of primary orbital Rhabdomyosarcoma through a representative case of a patient that has been managed at our institute as well as a review of the literature.
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