The yellow nail syndrome (YNS) is a rare disorder of unknown cause characterized by the triad of yellow and thickened nails, lymphedema and respiratory manifestations. We review the current state of knowledge, particularly regarding the diagnosis and management of this disorder. Available data suggest acquired lymphatic dysfunction to be the predominant mechanism underlying the clinical manifestations of YNS. The clinical features are variable among individuals diagnosed to have this disorder, and these features can vary over time. Although many disorders have been reported to be associated with YNS, there is no consistent theme in these associations. Longevity of patients with YNS is modestly reduced when compared with a control population. There is no specific treatment for YNS, but most patients can be managed with supportive measures aimed at ameliorating various clinical manifestations. The pathogenesis of YNS remains poorly defined. The diagnosis is established on the basis of characteristic clinical features including abnormal nails, lymphedema and respiratory manifestations. The clinical course is generally benign, and current treatment aims to control the various clinical manifestations of this obscure disease process.