Klippel-Trenaunay Syndrome (KTS) is a rare triad of congenital vascular malformations involving extensive Port wine stains, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation affecting limb, pelvicor abdominal organs. Pregnancy is known to exacerbate KTS complications and can put women at increased obstetrical risk due to deep venous thrombosis and other thromboembolic events. We report a case of a pregnant woman with KTS with the personal antecedent of two episodes of pulmonary thromboembolism.

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