Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge. Here, we report a patient who was only recently given the diagnosis of Schnitzler syndrome although the typical symptoms had been present for about 6 years. Administration of the B-cell antibody rituximab did not exert any beneficial effect despite effective elimination of B cells and a reduction of the paraprotein. In contrast, injection of the interleukin-1-receptor antagonist anakinra caused a complete remission within a few days.