Abstract. Primary pulmonary lymphangiectasia (PPL) is a rare developmental disorder affecting the pulmonary lymphatics, often present antenatally with pleural effusions and hydrops. Neonatal presentation is almost always fatal. It has been associated with Turner’s and Noonan’s syndromes, but the association of Trisomy 21 with PPL is rare. We report on a term infant with Trisomy 21 and pulmonary lymphangiectasia presenting as nonimmune hydrops and bilateral persistent chylothoraces.