Introduction: Rhabdomyosarcoma is an extremely aggressive malignant tumor that rarely occurs in adults. Its orbital location represents only 9% of all damage to the head and neck. Case presentation: We report the clinical case of orbital rhabdomyosarcoma in a 23-year-old male patient who presented with progressive right exophthalmos with cervical lymphadenopathy. The introduction of a treatment combining chemotherapy based on ifosfamide, vincristine and actinomycin, and orbital radiotherapy of 66 Gy according to the intensity modulation technique (IMRT), made it possible to obtain a complete remission with a 12 month follow-up. Conclusion: Orbital rhabdomyosarcoma is a rare tumor in adults. Early care improves the vital and functional prognosis.