目的　探讨先天性肺淋巴管扩张的临床及预后。方法　回顾性分析1例表现为胎儿双侧胸腔积液的先天性肺淋巴管扩张症患儿的临床资料。结果　患儿于胎儿期就表现为双侧胸腔积液，生后呼吸困难，双侧大量气胸、乳糜胸，低蛋白血症，持续性肺动脉高压。给予肺泡表面活性物质、NO吸入、高频机械通气、反复白蛋白输注，治疗3个月后出院。出院后第10天又以“重症肺炎、慢性肺病”收治入院，经机械通气、抗感染治疗2个月余，仍不能离氧，存活半年死亡。结论　先天性肺淋巴管扩张症罕见，产前诊断困难。对于生后呼吸困难严重、有明显间质性肺气肿表现的患儿，应适时给予肺组织活检或核素淋巴管显影扫描以明确诊断。

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.