<p>Congenital pulmonary lymphangiectasia is a rare condition caused by failure of pulmonary lymphatic vessels to develop normally. It is a known cause of non-immune hydops fetalis and congenital chylothorax. While it has been recognised in the literature since the 1950s, no standardised treatment has yet emerged and a definitive cause is still unknown. This review seeks to draw together current information on diagnosis, treatment and prognosis, and to recommend that a systemic evaluation of such would provide direction for clinicians in dealing with patients aᰀected by this condition.</p>
