Objective To evaluate the diagnostic value of vascular endothelial growth factor (VEGF)-D detection for the diagnosis of lymphangioleiomyomatosis (LAM) by Meta-analysis. Methods Literatures published before August 2017 were retrieved from PubMed, Embase, China Biology Medicine database, China National Knowledge Internet, Wangfang, and VIP database to retrieve the study about VEGF-D detection for LAM. The studies were screened according to the inclusive and exclusive criteria, the data were extracted, the quality was assessed and the Meta-analysis was performed with related statistical software. Results Six primary studies were included and 521 patients met the inclusion criterion. The Meta-analysis showed the pooled sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio of 0.75 [95% confidence interval (CI) (0.70, 0.79)], 0.95 [95%CI (0.91, 0.98)], 16.20 [95%CI (8.70, 30.19)], 0.20 [95%CI (0.10, 0.40)] and 89.49 [95%CI (38.46, 208.22)], respectively. The area under the curve was 0.953 9. Conclusions VEGF-D detection showes a good diagnostic value for LAM. A positive result is more clinical meaningful compared with a negative result, helping for the confirmation of the disease.