Objective: To assess any possible adverse effects of endometriosis on pregnancy outcome and complications, as well as in the postpartum period. Study Design: A systematic review of the literature. Data Sources: We searched all publications in Pubmed, Scopus and the Cochrane Library with the key words endometriosis, pregnancy outcome, preeclampsia, preterm birth, small-for gestational age babies (SGA) and postpartum. Eligibility Criteria for Selecting Studies: All studies reporting on endometriosis and perinatal outcome until December 2009. Results: According to the available bibliographic data, we found 38 reported cases of pregnancies, four being twin pregnancies, complicated by endometriosis (Table 1). Studies referring to specific pregnancy complications (preterm birth, small-for-gestational age babies, preeclampsia and postpartum complications) were limited to a total of 12 publications [61-63, 69, 75, 80, 82-86]. The few reported complications during pregnancy included hemoperitoneum and spontaneous bleeding [1, 9-21], perforations of the jejunum, appendix and sigmoid colon [22-24], urohemoperitoneum [25], deciduosis of the appendix [26], deciduosis of the omentum [27], infected endometrioma [28], hemoperitoneum and hemothorax [29], catamenial pneumothorax [30], endometriosis imitating a bladder tumor [31], decidualization mimicking ovarian malignancy [32], rupture of ovarian endometriotic cyst [23, 33], and rupture of the uterus affected by endometriosis [34] (Table 1). Data regarding the effect of endometriosis on preterm birth, small-for gestational age babies and preeclampsia, both in spontaneous pregnancies as well as in those conceived by Assisted Reproductive Techniques (ART) were conflicting. Conclusions: Symptoms of endometriosis often disappear during pregnancy. Yet, endometriosis must be included in the differential diagnosis of hemoperitoneum presenting during pregnancy or of heavy vaginal bleeding postpartum. Both maternal and fetal morbidity and mortality can be quite high and the woman’s childbearing ability might be irreversibly affected. The few available data on the association between endometriosis and preeclampsia are at present controversial. Together with the studies reporting an increased risk for preterm birth in women with endometriosis, physicians must be aware that close antenatal follow-up and early diagnosis is crucial. Postpartum manifestations of endometriosis, although extremely rare, can give rise to severe complications with a high possibility of irreversibly affecting the woman’s childbearing ability. Keywords: Endometriosis, postpartum, preeclampsia, pregnancy outcome, preterm birth, SGA (small for gestational age)