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.

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