介绍６例少见先天性畸形——肺淋巴管扩张症。肺的特征性改变为肺呈蜂窝状，肺膜下、肺小叶间隔内、支气管周围和血管周围结缔组织内淋巴管极度扩大。其壁很薄，仅以内皮细胞围成。扩张的淋巴管可能由于先天性发育缺陷或由于淋巴管主干梗阻，但仅表现于肺静脉高压时。 Six cases of congenital anomalies - pulmonary lymphangiectasis were introduced. The characteristic changes of the lungs were honeycombing of the lungs, and the lymphatic vessels within the connective tissues around the bronchus and peri-sub-lung, within the lobules, and the perivascular tissues were extremely enlarged. The wall is thin, surrounded by endothelial cells only. Expansion of the lymphatic vessels may be due to congenital defects or due to lymphatic trunk obstruction, but only in the pulmonary hypertension.