Angiomyolipomas are frequent tumours of the kidneys. They are very important in the differential diagnosis of other kidney tumours; sometimes they can present a large size and manifest as an acute massive retroperitoneal haemorrhage. They generally should not be treated unless there are life-threatening problems. In this case report, we present an acute surgical condition due to rupture and haemorrhage of a giant angiomyolipoma. In addition, we review the literature on angiomyolipomas and their severe complications in order to help young surgeons who may be involved in such difficult and life-threatening cases as ours.