A case of a 54-year-old woman exhibiting the yellow nail syndrome with exudative pleural effusions, lymphoedema, pericardial effusion, rhinosinusitis and bronchiectasis is described. This case illustrates the rather benign course of the syndrome over ten years. A review of the literature is provided. The etiology of the syndrome is obscure, while the pathogenesis seems to involve impaired lymphatic drainage. The entity should be considered in the differential diagnosis of bilateral pleural effusion. It is likely that the condition is not as rare as it appears from the number of published reports, and a greater awareness of its various forms will probably lead to more frequent diagnosis.