Background: Pregnancy-induced pelvic and extra-pelvic cavernous hemangiomatosis is a serious condition, associated with considerable maternal and fetal risks. This report describes the ultrasound (US) features and the clinical management of such rare condition in a young caucasian woman. Case: A 20-year-old woman was referred to our department following the occurrence of swelling in her inguinal and vulvar area together with lipotimic episodes. Diffuse cavernous hemangiomatosis of the pregnant uterus associated with vaginal, inguinal and vulvar varicosities was diagnosed clinically and by 2D and 3D US. The patient underwent expectant management of the gestation, under close US monitoring of both superficial and inner varicosities, careful surveillance of the maternal and fetal condition and prophylaxis for thrombotic complications with medical therapy. A cesarean section was performed at 37 weeks of gestation because of the worsening of her lipotimic episodes and her unfavorable Bishop’s score. Conclusion: This is the first report in which pregnancy-induced varicose disease involved contemporarily uterine, vaginal, inguinal, and vulvar veins totally sparing the lower extremities. This case suggests that, under close monitoring, a conservative approach can be adopted in such conditions. Vaginal delivery is to be preferred, but if cesarean section is required, the surgery should be performed under general anesthesia and packed red cells and plasma units should always be available.