We report on a 45-year-old female who presented with end-stage renal d
isease due to Wegener's granulomatosis limited to the kidneys. Ultraso
nography and computed tomography revealed a masslike expansion involvi
ng the upper pole of an otherwise small right kidney. Histopathology o
f a nephrectomy specimen revealed cystic dilatation of renal tubules a
nd active lesions of Wegener's granulomatosis in the upper pale. The l
ower part of the kidney showed atrophic changes. Wegener's granulomato
sis presenting as a renal mass is extremely rare. Only 3 cases have be
en previously reported in the literature. Wegener's granulomatosis onl
y limited to the kidney is also race. 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 p
atient continues to be free of any extrarenal involvement 18 months fo
llowing presentation, although subsequent development of extrarenal le
sions cannot be ruled out.