An increasing number of women with severe endometriosis have conceived through assisted reproductive technology, but endometriosis can cause life-threatening complications for both the mother and baby during pregnancy. We describe a case of endometriosis-induced spontaneous ileal perforation in pregnancy with massive intestinal haemorrhage that required caesarean section, right hemicolectomy and terminal ileum resection at 33 weeks of gestation. Spontaneous perforation associated with intestinal endometriosis in pregnancy is a rare complication, and only seven cases have been reported in the English literature. To the best of our knowledge, this is the first report of spontaneous ileal perforation due to endometriosis. Moreover, this is probably the first case of massive intestinal haemorrhage in pregnancy that resulted from intestinal endometriosis. These risks must be explained to couples suffering from endometriosis-related infertility prior to conception by assisted reproductive technology, and multidisciplinary management may be mandatory for women with severe endometriosis in pregnancy.