Vasculitic syndromes are a group of disorders characterized by inflammation of the blood vessels. They include polyarteritis nodosa (PAN), Churg-Strauss syndrome (CS), and Wegener granulomatosis (WG) that differ on type, size, and location of the involved vessels. We report a rare case of granulomatous vasculitis leading to ureteral stenosis in the absence of other systemic manifestations of the disease. The patient was a 56-year-old woman who began to complain of left lumbar pain, nocturia, and dysuria. Computed tomography scan demonstrated a large abdominal mass with ureteral compression. A tissue specimen obtained by laparotomy revealed the presence of a chronic inflammatory process with fibrosis, granulomatous reaction, extravascular eosinophilia, and prominent vasculitis. She was put on corticosteroids and azathioprine with a decrease of the tumoral mass. This incompletely classified vasculitis was an unexpected cause of the abdominal mass.