We report on a 45-year-old female who presented with end-stage renal disease due to Wegener’s granulomatosis limited to the kidneys. Ultrasonography and computed tomography revealed a masslike expansion involving the upper pole of an otherwise small right kidney. Histopathology of a nephrectomy specimen revealed cystic dilatation of renal tubules and active lesions of Wegener’s granulomatosis in the upper pole. The lower part of the kidney showed atrophic changes. Wegener’s granulomatosis presenting as a renal mass is extremely rare. Only 3 cases have been previously reported in the literature. Wegener’s granulomatosis only limited to the kidney is also rare. A brief review of 23 previously reported cases who initially presented with renal disease is presented. All but 2 patients subsequently manifested extrarenal lesions. Our patient continues to be free of any extrarenal involvement 18 months following presentation, although subsequent development of extrarenal lesions cannot be ruled out.