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Developmental abnormalities of the spleen are common, much less common is splenic fusion abnormality. It may present as a renal mass sometimes with symptoms of hypersplenism (anemia) or as a renal mass only, mimicking primary or secondary renal neoplasms on imaging studies. Its documented that occurred on the right side which is significant that provides evidence for the possible migration of spleen cells in embryogenesis as an explanation for some other splenic fusion anomalies.We report a case of splenorenal fusion in a 5-year old girl with only right functional kidney who initially presented with a large renal mass which mimicked a primary renal neoplasm with no history of splenic trauma or splenectomy in past medical history. Also, the child didn't present with symptoms of hypersplenism. The child underwent right nephrectomy for a renal mass that was subsequently confirmed as right splenorenal fusion pathologically. Keywords: Splenorenal fusion, Renal mass, Extramedullary hematopoiesis, Child