Abstract: The main pathological types of malignant transformation of endometriosis are mullerian epithelial carcinoma, endometrioid stroma sarcoma, etc., and the most common entities in ovary are endometrioid carcinoma and clear cell carcinoma. The development of endometriosis-related ovarian cancer may involve various molecular pathways, such as Wnt/β-catenin signaling pathway, MAPK signaling pathway, PI3K/Akt/mTOR signaling pathways and so on. The synergy effect among key genes on pathways, sucn as β-catenin, MAPK, PIK3CA, ARID1A and others, activates the corresponding downstream signaling pathways, and promotes tumors development. Herein, the malignant transformation of endometriosis and its underlining molecular mechanism, the clinicopathological characteristics of endometriosis related ovarian malignancies will be reviewed and discussed.