Background:Schnitzler syndrome encompasses monoclonal gammopathy, urticaria, inflammation, recurrent fever, bone pain and arthralgia, with occasional lymphadenopathy and/or hepatosplenomegaly. It is a rare condition with approximately 100 cases reported in the literature. To our knowledge, this is the first reported case of cold-induced physical urticaria with Schnitzler syndrome.Main observations:A 43-year-old woman presented to an allergy and immunology clinic with a 7 year history of chronic urticaria, angioedema with anaphylaxis, monoclonal gammopathy and bone pain. Her urticaria was triggered by cooler temperatures and an ice cube test for cold induced urticaria was positive. In spite of aggressive therapies this patient remains symptomatic.Conclusions:Schnitzler syndrome is under-recognized, particularly variants of the classical description of Schnitzler syndrome. Other diseases, especially those of hematologic origin, should be ruled out. This condition is largely refractory to conventional therapies, although novel treatments, such as interleukin-1 receptor inhibitor, may show promise.