Primary renal extraskeletal Ewing sarcoma (EES) is rare but well known to be aggressive, less responsive to the treatment, and has early predilection for metastases. Metastases at the time of diagnosis to the lungs or bones are associated with poor outcome. We present a case of primary renal EES in 57-year-old woman with multiple metastases to the lungs at the time of diagnosis with complete remission of the disease for the last 8 years following multimodality treatment Multidisciplinary approach for the management of EES has definitely improved the quality of life and the survival of the patients.