The pathogenic mechanisms underlying Lyme disease remain uncertain but an increasing number of reports suggest a vascular inflammatory process. On the other hand, the so-called systemic vasculitides, even though they remain of pathological definition, have recently been characterized by the presence, in the serum, of anti-neutrophil cytoplasmic antibodies (ANCA). We report on a patient, finally diagnosed as having neuroborreliosis, who presented initially with multiple mononeuropathy, ANCA, vascular lesions at muscle biopsy and lymphocytic meningitis. Despite antibiotherapy, he presented with two recurrent strokes, from which he completely recovered. He also developed oto-sinusitis. The disease was finally clinically and biologically controlled by immunosuppressive therapy. The relationship between angiitis and Lyme disease are discussed. It is hypothesized that Borrelia burgdorferi infection may cause a systemic vasculitis following its own course and requiring its own treatment.