OBJECTIVE: To perform a review of Tuberous sclerosis complex and its complications during the pregnancy from up to date scientific literature. DESIGN: Complex systematic review of the literature. SETTING: 2nd Department of Obstetrics and Gynaecology, University Hospital Bratislava, Comenius University, Bratislava, Slovak Republic. METHODS: Complex analysis of the syndrome, systematic search of MEDLINE and Slovak Medical Library. The reason to create a complex review of this syndrome was our clinical experience with one of the most acute lifethreatening condition of this syndrome - acute retroperitoneal haemorrhage in the pregnancy. CONCLUSION: Tuberous sclerosis can cause a rare, but potentially lifethreatening complications, especially during the pregnancy. These should be carefully dispesarized and in complicated cases acute radical management should be considered.