Orbital rhabdomyosarcoma is the most common malignant orbital tumor of child-hood originating from mesenchymal cells. The presenting symptom is usually acute onset unilateral proptosis. The rapidly progressive course of the findings may resemble infectious and inflammatory orbital diseases. Radiological imaging and histopathological examinations are crucial for differential diagnosis. The main goal of treatment with a multidisciplinary approach is to control both local and distant spread of the tumor and to prevent further damage. With the introduction of chemotherapy and radiotherapy in the treatment, the overall survival rate has in-creased. Thus, aggressive surgical approach for complete removal of the tumor has been abandoned.