Orbital rhabdomyosarcoma is the most common soft tissue malignancy in children. Orbital rhabdomyosarcoma accounts for 10% of all rhabdomyosarcomas in the body. Distant metastases from orbital rhabdomyosarcoma account for 3% of the children seen. The pathology of orbital rhabdomyosarcoma is mainly embryonal, glandular, and pleomorphic. A multimodal combination of surgery, radiotherapy, and combined chemotherapy is the current classical treatment approach. Prior to the 1960s, total orbital content removal was the effective treatment, and about 30% of patients were cured. The aim of modern surgery is to control the tumor while preserving the function and appearance of the child’s eye, so the main goal of surgery today is to obtain pathology, and it is not necessary or possible to remove all of the tumor. After 1960, the tumor disappeared after the child received radiation therapy. Since then, it has been recognized that rhabdomyosarcoma is sensitive to radiation therapy. Currently, only chemotherapy is used for those embryonic tumors that show negative surgical margins. Radiation therapy is mandatory for partial resection and biopsy, and for those whose surgery does not provide a margin situation. The dose of radiotherapy is 45-54 GY, but the damage to the eye is inevitable and clinical studies are now focused on combining chemotherapy to reduce the dose of radiotherapy. With current advances in radiotherapy technology, it is possible to completely destroy the tumor while avoiding radiation therapy damage. Chemotherapy is equally sensitive to rhabdomyosarcoma. The current literature shows a complete remission rate of 70% with chemotherapy. However, 45% of patients recur after chemotherapy alone. The role of chemotherapy is to be used at the right time and to avoid the side effects of radiation therapy. However, more chemotherapy then receives more chemotherapy damage, mainly to the reproductive organs of children and the possibility of hematologic tumors. Likewise relapse retreatment after chemotherapy alone increases the financial burden of the family.