Yellow nail syndrome (YNS) is a rare disorder of unknown cause associated with yellow nails, lymphedema and respiratory manifestations. It was first described by Samman and White in 1964, and to date, approximately 150 cases have been reported. The diagnosis of YNS is essentially a clinical one and based on the presence of characteristic findings. We report a case of YNS of a 62-year-old female who presented with a 4-month history of dyspnea and recurrent pleural effusion. The patient had a 5-year history of leg swelling and dyspnea. She had been managed with medications for congestive heart failure (CHF) for two years and she was referred to our hospital for further evaluation and management.