We describe a 36-year-old patient with an aggressive, midline intranasal and naso- and oropharyngeal destructive process. For months the patient denied heavy abuse of nasal cocaine, but finally admitted it. Necrosis and atrophy of the inferior and middle nasal turbinates bilaterally, prominent naso and oropharyngeal ulcers, nasal septal as well as hard palate perforation were observed clinically. Repeated biopsies revealed focal areas of chronic inflammation and necrosis, but there was no evidence of vasculitis or granuloma formation. Since serum was slightly positive for antineutrophil cytoplasmic antibody, the initial diagnosis was Wegener's granulomatosis. In the United States there have been a few reports on a new cocaine-associated syndrome presenting as an aggressive, midline, intranasal and intrapharyngeal destructive process mimicking limited Wegener's granulomatosis and midline reticulosis. We report the first such case in Europe and offer guidelines for the diagnostic work-up of such cases.