Context: Klippel-Trenaunay syndrome (KTS) is a congenital disease and usually shows two major features: vascular malformations and localized disturbed growth of bone or soft tissue. Due to the thromboembolic disease risk and bleeding is frequent, obstetric and anesthetic management in pregnancy with KTS can be quite complex. Objective: We aimed to report and discuss a rare case of pregnant KTS in our clinic. Case: 22 years old, 36th-week gestation, primigravida patient applied to our clinic. She had no family history of KTS and no thrombosis history.Multiple vaginal venous varicosities, common cervical haemangiomas, hypertrophied left leg and arm, atypical localized varicose veins in the left leg, five cm longer left leg than right leg, and sharply demarcated by dark and light red face and hands were observed on physical examination. After five days of hospitalization, uterine contractions and cervical dilatations began. Cesarean section was performed because of many varicose veins in the vulvovaginal region.The patient didn't have MRI for excluding possible varicosities in the spine, so spinal anesthesia can not be performed. In the C-section, there weren't any varicose veins either on the pelvic peritoneum or uterus. There were no complications in the postpartum period. Conclusion: Because of the physiological changes that occur in pregnancy, the obstetric and anesthetic management of CTS can be quite complicated. The multidisciplinary approach is needed in these patients.