A newly recognized disease is described, with a report of one case and review of sixteen others in the literature presented under various names. It occurs in female subjects, is characterized clinically by respiratory insufficiency and often by chylothorax. The lungs have increased smooth muscle and multiple cysts, but this is a unique cause of "honeycomb lung" and should not be confused with the numerous other causes of muscular hyperplasia and/or cysts of the lung. The other distinguishing feature is the involvement of mediastinal and retroperitoneal lymphatic tissue. The possible etiologic and therapeutic implications are discussed.
