目的探讨先天性肺囊肿的临床及病理特点。方法经手术及病理确诊为先天性肺囊肿患儿29例,回顾性分析其临床及病理资料。结果临床首发症状多出现于不足6个月(34.5%)的婴幼儿,以发热伴咳嗽、呼吸困难及局限性固定湿罗音或呼吸音减低为临床特征;病变部位以右肺居多,以两肺下叶受累居多。临床症状不典型,误诊率高。结论临床上应当重视先天性肺囊肿的早期诊断和早期手术治疗。 Objective To investigate the clinical and pathological features of congenital pulmonary cysts. Methods Twenty-nine children with congenital pulmonary cysts were confirmed by operation and pathology. The clinical and pathological data were retrospectively analyzed. Results The first symptom of clinical manifestations occurred in less than 6 months (34.5%) of infants and young children with fever and cough, dyspnea and limitations of fixed wet rales or reduced breath sounds as the clinical features; the lesion to the right lung mostly to two Lower lung involvement most. Clinical symptoms are not typical, high misdiagnosis rate. Conclusions The early diagnosis and early surgical treatment of congenital pulmonary cysts should be paid more attention in clinical practice.