Abstract
Ewing sarcoma is a small round cell tumor of uncertain differentiation, primarily originating in bone in children and adolescents. Ewing sarcoma of the kidney is a rare occurrence and follows an aggressive course with early metastasis. Herein, we present a case of a 16-year-old male presenting with abdominal pain and lump. He underwent nephrectomy and histopathological diagnosis of small round cell tumor with differential diagnosis of Ewing sarcoma was made which was further confirmed by immunohistochemistry. Thus, Ewing sarcoma must also be taken into consideration while dealing with tumors of the kidney in young age group.