The yellow nail syndrome is a rare disease. The diagnosis is clinically based on three features: yellow discolouration on the nails, chronic lymphedema, and pleural effusions. The treatment of the yellow nails is controversial. Spontaneous healing is reported in 30% of the cases. Vitamin E can otherwise be tried. The syndrome is a benign disease but the literature describes an association to malign and autoimmune diseases. It is therefore important to investigate the patients with this syndrome. The present case story shows the development of yellow nail syndrome in a 73 year-old man.