The aim is to perform a literature search on the role of pregnancy in the rupture of renal angiomyolipoma. Articles published from 1952 to 2004 in the Medline database were searched using the keywords renal angiomylipoma and pregnancy. Pathologies associated with angiomylipoma (lymphangioleiomyomatosis and Bourneville tuberous sclerosis) were taken into account. Seventy-two cases of association of renal angiomyolipoma and pregnancy were found, out of which 58 presented a haemorrhage. In only 26% of the cases, renal angiomyolipoma had been documented prior to pregnancy. Clinical presentation was similar to cases occurring among non pregnant women: abdominal pains (88%), hypotension or shock (33%) and hematuria (24%). Average size of the rupture was 11,7 cm. Rupture does not occur solely with the first pregnancy but occurred equally during the first, second and third pregnancy. Average gestation age upon occurrence of haemorrhage was 27 weeks with a minimum at ten weeks. Therapeutic strategies at the time of shock or hypotension were total nephrectomy in 79% of the cases, 7% polar nephrectomy, 7% embolisation followed by nephrectomy and 7% abstention. A causal role of pregnancy in the atraumatic rupture of angiomyolipoma is not clearly defined. Nevertheless, many arguments (whether it be abdominal mechanical pressure, hormonal or histological ones) suggest that a pregnancy could increase the risk of renal angiomyolipoma rupture. These patients should have a medical follow-up period at closer intervals during their pregnancy and the postpartum phase.