History and Objectives: Yellow nail syndrome along with pleural effusion and lymphatic edema, with positive family history has been reported. A case familial yellow nail syndrome in the department of internal medicine of Shaheed Beheshti hospital of Kashan in 1996 is presented.

Patient’s profile: A 70 year old woman with yellow nail syndrome and right side pleural effusion and lower extremity edema was followed up and treated for 1.5 years. Her family and her grand children were examined. She reported an eight years of asthma history. Chylous ascite fluid aspiration, investigation of intensity of edema and pulmonary CT-scan were performed. In examining her nails, changes in shape, onycolysis and dystrophy of all nails of hand and foot were observed. CT-scan showed bronchiectasias of medullary lobus inferior of right pulmonary was observed. Chylous ascite fluid was transudative and intensity of edema was due to hypoalbuminemia.

Family study: Patient has four children (3 boys and 1 girl). Dystrophy, changes in color and shape of nails of hand and foot, along with lower extremity edema were observed in two boys and one girl. One of the boys had asthma. Other family members and patient’s grand children were healthy. Patient reported similar nail abnormalities and lower extremity edema in her own grandmother.

Conclusion: The present report show a case of yellow nail syndrome and implication of genetic factors pathogenesis of the disease. Therefore it essential that family history is considered in the characterization of the disease.