We treated a patient with an atypical presentation of Wegener's granul
omatosis (WG) with dural involvement as the initial clinical manifesta
tion. A 37-year-old man had a dural lesion without lower respiratory t
ract or renal manifestations in the initial clinical course, His only
initial symptom was headache, and at disease onset computed tomography
(CT) and magnetic resonance imaging (MRI) of the head revealed bilate
ral abnormal subdural masses, The diagnosis of WC was made based on th
e results of needle biopsy of the nasal polyps and the finding of posi
tive circulating antineutrophil cytoplasmic antibodies (c-ANCA). He ac
hieved remission on daily prednisone and cyclophosphamide with the lat
er addition of sulfamethoxazole-trimethoprim.