Renal medullary carcinoma is a highly aggressive neoplasm of the kidney that originates from renal medulla and affects most commonly young adults and can also occur in children. The presentation is usually with hematuria, abdominal pain and less commonly with weight loss, fever and abdominal mass. The prognosis is dismal. The mean survival from time of the diagnosis is 3 months. A child with renal medullary carcinoma presenting with abdominal mass is described because this tumor is very rarely encountered in childhood and can be confused with yolk sac tumor histopathologically.[Turk J Cancer 2005;35(2):96-98].