We report a case of chronic idiopathic recurrent urticaria-angioedema and gastroesophageal reflux disease in a 35-years-old man, followed after 2 years by Raynaud's phenomenon and esophageal dysphagia, recurrent cough and dyspnoea, and after 4 years by systemic sclerosis. A review of the literature and possible correlated pathogenetic mechanisms are presented.