BACKGROUND Pyoderma gangrenosum (PG)-like lesions can be associated with Wegener's granulomatosis (WG); in rare instances, they could be the presenting sign of WG. The purpose of this study was to elucidate the relationship between these two disorders. METHODS The clinical and histologic data from a case of WG with primary ulcerative lesions of the face were compared with five other cases previously reported in the literature. RESULTS A predominance among young men was noted. In three patients the histology of the skin ulcerations was suggestive of WG, in the remaining three, including the case described, it was nonspecific. Two cases developed pulmonary WG after the skin lesions cleared, showing that the extent and severity of skin lesions do not necessarily correlate with the systemic disease. CONCLUSIONS When the histology of the skin is nonspecific, the diagnosis of PG or PG-like lesions of the head and neck should not be considered definitive and the patients should be carefully monitored to rule out the possible presence of WG.