Recurrent attacks of pulmonary atelectasis were the leading sign of familial Mediterranean fever in a young man of Jewish-Georgian extraction. His mother suffered from the more common manifestations of the disease. Treatment with colchicine caused a complete disappearance of his attacks. However, when challenged by discontinuing colchicine therapy for eight days, another, documented attack of pulmonary atelectasis occurred. To our knowledge, this is the first case of familial Mediterranean fever presenting with recurrent pulmonary atelectasis.