Introduction: The most common childhood orbital malignancy is the rhabdomyosarcoma contributing almost 10% of all RMS cases. The mean age of orbital RMS is 6-8 years and rare case of RMS from birth to the 8th decade was reported. Male are more affected as compared to female. Total 60.4% male were affected with RMS as compared to female was 39.6%. Objective: The objective of the current study was to describe a case of recurrent orbital rhabdomyosarcoma and effectiveness of chemotherapy to RMS in a 2 year old child. Methods: Computer tomography, magnetic resonance images and excision biopsy were done for confirmation of tumor. After confirmation patient was treated with chemotherapy. Results: Excision biopsy showed tumor composed of round blue cells with high nodular cells ratio and hyper chromatic nuclei and finding also presented favors of Embreyonal rhabdomyosarcoma and child was treated with chemotherapy after completion of chemotherapy course this little child was came to his normal life but after 6 months sudden symptoms of recurrence has been appeared and this time tumor was most aggressive and cannot be controlled with only chemotherapy treatment. Conclusion: To achieve a good survival rate of RMS in children and elderly age there is a need to complete surgical tumor resection in anywhere of the body. Only chemotherapy should not be helpful and cannot be preventable from recurrence. Early diagnosis and stared treatment is the best approach and any delay in diagnosis can have a negative effect on health status.