The incidence of renal angiomyolipoma (RA) is 0.3% in the general population, and even more infrequent during pregnancy. Pregnancy can increase the risk of rupture, although the causal mechanism is still not clearly defined. We completed a Medline literature search for articles on RA and pregnancy and its complications. We identified 16 articles (all case reports), but selected only 13 because of unavailable data in the 3 other articles. We report the case of a 30-year-old primiparous woman who presented at the emergency ward with a non-reassuring pattern at fetal monitoring; an urgent cesarean section was decided and carried out. After surgery, a wide retroperitoneal hematoma was observed caused by the rupture of an RA. Conservative management by means of arterial embolism was done and the patient was discharged on postoperative day 10. RAs seem to have a higher risk of rupture during pregnancy, but they should be managed conservatively when hemodynamically possible. Individualization of each case is necessary in order to achieve the best outcome for both the mother and fetus.