Abstract: Objective: To investigate the diagnostic value of chest CT and CT lymphangiography (CTL) in congenital pulmonary lymphangiectasia (CPL). Materials and methods: The clinical and imaging datas of 41 patients with CPL diagnosed by clinical and imaging from August 2012 to July 2021 were retrospectively analyzed. All patients underwent chest CT examination before direct lymphangiography (DLG) and CTL after DLG. The CT and CTL imaging manifestations were analyzed and statistically described using the composition ratio in the categorical variable data. Results: Among the 41 patients, CT showed thickening of bronchovascular bundles in 33 cases, interlobular septal thickening in 32 cases, intralobular interstitial thickening in 4 cases, and ground-glass opacity in 26 cases, 5 cases of lung consolidation, 14 cases of atelectasis, 24 cases of pleural effusion. CTL lipiodol was abnormally deposited at the end of the thoracic duct in 35 cases, bronchovascular bundles in 31 cases, hilum in 32 cases, pleura in 17 cases, mediastinum in 36 cases, pericardium in 17 cases, diaphragm in 3 cases, intraperitoneal in 10 cases, and retroperitoneal in 10 cases , 20 cases of pelvis and perineum. Conclusion: CT and CTL can show the extent and distribution of Lymphangiectasia, and provide imaging basis for the diagnosis and treatment of CPL.