The aim of this study was to examine the prevalence of anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Behcet's syndrome (BS) with large vessel involvement. The sera of 48 consecutive patients with BS with large vessel involvement, seven patients with Wegener granulomatosis (WG), and 10 healthy staff were studied for the presence of the cytoplasmic (c) and perinuclear (p) pattern of ANCA by indirect immunofluoresance (IIF). The sera were also examined for antibodies against proteinase 3 (anti-PR3) and myeloperoxidase (anti-MPO) by enzyme-linked immunosorbent assay (ELISA). None of the sera from the patients with BS and healthy controls had detectable ANCAs, while all positive control sera from patients with WG showed ANCA positivity in some form. This study confirms that there are no detectable ANCAs in patients with BS with large vessel involvement by IIF and ELISA tests.