Granulomatosis with polyangiitis (Wegener's granulomatosis) is one of the idiopathic immune-mediated small-vessel vasculitides described in humans which are characterised by the presence of circulating antineutrophil cytoplasmic antibodies. It most commonly involves capillaries, venules and arterioles of the ear, nose and throat, lungs and glomeruli. A case of destructive haemopurulent rhinitis associated with relapsing periods of pyrexia, lethargy and stiffness as well as generalised pulmonary infiltrates in a young Anatolian shepherd dog is presented that closely resembles granulomatosis with polyangiitis (GPA) as reported in humans. Perinuclear antineutrophil cytoplasmic antibodies (pANCA) were detected in the dog's serum. Signs resolved promptly and completely once immunosuppressive doses of prednisone were administered, and have not recurred. This is the first report on the use of pANCA to investigate rhinitis in dogs. It is also, to the authors’ knowledge, the first description of a relapsing haemopurulent lytic rhinitis in this species. The concurrent manifestations of erosive haemopurulent rhinitis, ground-glass opacities on pulmonary computed tomography, pyrexia and listlessness resemble GPA as described in humans.