Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. Treatments for lymphangiectasia have included corticosteroids for patients with primary inflammatory conditions, dietary modifications, surgical resection for isolated lesions, octreotide, antiplasmin therapy and fibrin glue pleurodesis. However, there is no experience with pleurodesis by autologous blood therapy in the literature. We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of life and improved with pleurodesis by autologous blood therapy. Copyright (c) 2007 S. Karger AG, Basel.