Wegener's granulomatosis (WG) is a systemic disease well recognized by physicians. The upper and lower respiratory symptoms associated with the disease are easily recognized; however, its presentation as a solid renal mass is underappreciated. We present a case of a 61-year-old man who presented with a 5.2-cm renal mass diagnosed as WG after nephron-sparing surgery. The patient presented with mild symptoms suggestive of WG, but the antineutrophil cytoplasmic antibody test was negative. The renal mass and concerns about possible malignancy obscured the diagnosis of WG. This case illustrates that WG should be considered in the differential diagnosis of solid renal masses.