Background: To review women with symptomatic and clinically progressive endometriosis after menopause in the absence of estrogen intake or excessive systemic endogenous production.Design: Seven case reports and a systematic review of the literature from 1995 till February 2018. Results: Only 7 case reports from the authors and 29 cases from the literature described women with either cystic ovarian or deep endometriosis. Severity, symptoms, and localization are highly variable. No case report describes symptomatic superficial typical lesions. In 22 of 36 women (61%), symptoms started more than 10 years after menopause. Conclusions: Symptomatic and clinically progressive endometriosis after menopause in the absence of increased systemic estrogen concentrations or exogenous estrogen intake starts more than 10 years after menopause in the majority of women. This observation suggests that a genetic and/or epigenetic incident caused estrogen-independent progression, increased sensitivity to estrogens or increased local production of estrogens. This observation is important for understanding the pathophysiology of endometriosis, for the management of postmenopausal endometriosis, and for individualization of medical therapy of endometriosis since estrogen-independent endometriosis growth probably also occurs before menopause.