Ossifying renal tumor of infancy ORTI is a benign neoplasm, which presents with gross hematuria and less frequently as an abdominal mass, histologically it comprises a large cell with an epithelial nature and osteoid formation. We report a case of a 10-month-old girl who developed ORTI as non-opacified upper calyces of left kidney on excretory urography. We outlined the calcific and tumors nature of the lesion by ultrasound and computed tomography. The macroscopic and histological features were pathognomonic.