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Schnitzler’s syndrome is a rare and devastating autoinflammatory syndrome which can be difficult to diagnose. We describe two cases presenting with signs and symptoms consistent with Schnitzler’s syndrome with initial or continued absence of monoclonal gammopathy. Both patients responded to anti-IL-1β therapy, consistent with what is known for this disease. From our review of the literature, there has only been one other case of Schnitzler’s syndrome without a monoclonal gammopathy documented. Understanding that monoclonal gammopathy may not always be present is important to avoid delay in diagnosis and initiation of proper treatment.