Abstract: Objective　To summarize and review the clinical characteristics of congenital pulmonary lymphangiectasia with fetal bilateral pleural effusions. Methods　The clinical data of a newborn diagnosed as congenital pulmonary lymphangiectasia with bilateral pleural effusions in Obstetrics and Gynecology Hospital, was summarized. The clinical, radiographic features, treatment and prognosis of this case are discussed in the context of the literature review. Results　The premature infants presented with rapid progression bilateral pleural effusions, respiratory distress, chylothorax, hypoalbuminemia and persistent pulmonary hypertension at birth. The pulmonary surfactant was given and mechanical ventilation was used for respiratory support. NO was inhaled, high-frequency mechanical ventilation was applied and albumin was repeatedly administered. After treatment for 3 months in the NICU, the patient was discharged. After 10 days, the patient was administered to the PICU with "severe pneumonia, chronic lung disease, mechanical ventilation" and anti-infection treatments were applied for 2 months. After living for six months, the baby died. Conclusions　Congenital pulmonary lymphangiectasia is extremely rare and prenatal diagnosis was difficult. The disease should be considered in patients presented with progressive dyspnea, interstitial emphysema after birth. Lung tissue biopsy and radioisotope scanning should be performed in time to get diagnosis.