Description:
<jats:title>Abstract</jats:title><jats:p>Primary pulmonary lymphangiectasis (<jats:styled-content style="fixed-case">PPL</jats:styled-content>) is a rare congenital developmental abnormality of the lung with a generally poor prognosis. Only a limited number of patients with neonatal‐onset <jats:styled-content style="fixed-case">PPL</jats:styled-content> have been reported to survive. We present the case of a male preterm infant (gestational age 34 weeks 6 days) with histologically confirmed <jats:styled-content style="fixed-case">PPL</jats:styled-content>, complicated by hydrops fetalis, bilateral hydrothorax (treated <jats:italic>in utero</jats:italic> with pleuro‐amniotic shunts), and immediate respiratory distress at birth. He survived after extensive neonatal intensive care therapy and was discharged home at the age of 7 months. At last follow up he was 3 years 7 months old, still requiring assisted ventilation via tracheostomy, having recurrent episodes of wheezing and had mild global developmental delay. This case demonstrates that survival beyond the neonatal period is possible even with severe <jats:styled-content style="fixed-case">PPL</jats:styled-content> but long‐term morbidity may be relevant, and multidisciplinary management and close follow up are essential.</jats:p>