Yellow nail syndrome (YNS) is an uncommon condition characterized by a triad of yellow nail coloration, lymphedema and respiratory tract involvement. This syndrome typically affects middle-aged persons. Although several etiologies have been described, to date; the exact etiology remains unclear. Different treatment plans have been suggested, but all data available emphasize the fact that treatment is mainly symptomatic and the underlying disease is not targeted. The most reported treatment protocol is chemical pleurodesis combined with alpha-tocopherol (vitamin E). Hereby, we describe a case of YNS in a 34 year-old woman with the onset of symptoms in childhood. The symptoms improved dramatically after treatment with octreotide.