Background:
Klippel-Trenaunay-Weber syndrome is a rare neurocutaneous syndrome with vascular involvement. Given the rarity of the syndrome, its management in pregnancy is based on the outcome of a few case reports and expert opinion.
Case summary:
The management of a complicated case with its antepartum, intrapartum, and postpartum concerns has been addressed in this review.
Conclusions:
Prenatal consults with anesthesia, general surgery, intervention radiology, and internal medicine should be arranged, prior to delivery in anticipation of all the possible complications. Apart from the pregnancy management, preconceptional counselling including the genetics, prognosis, and contraception has an important role in patient management.