Endometriosis is a common condition in women of reproductive age. According to several epidemiological studies，endometriosis may be associated with various malignancies，but the frequency of malignancy arising in endometriosis is always supposed lower than 1%. About 80% of endometriosis-associated malignancy（EAM） have been found in the ovary，whereas 20% are localized in extragonadal sites like intestine，rectovaginal septum，abdominal wall，pleura and others. Once the endometriosis is malignant，it will pose a great threat to the health of patients. Currently，exploring effective methods for diagnosis and treatment of EAM is the major clinical challenge. The article reviews the studies of the risk factors，clinicopathological characteristics and pathogenesis of EAM.