||Klippel-Trenaunay Weber Syndrome (KTWS) is a rare congenital neuroectodermal disorder characterized by a triad of varicosities, cutaneous capillary abnormalities, and soft tissue hypertrophy in affected limbs. KTWS is often associated with arteriovenous malformations.1,2 The syndrome has been linked to genetic mutations in angiogenic factors that have not yet been well defined. A 15- year old patient with KTWS that underwent repair of a left radicular arteriovenous fistula originating at T11-L2 and the anesthetic challenges involved with her care is described in this case study. Her medical history was significant for capillary malformations, multiple arteriovenous fistulas of the left lower extremity and significant soft tissue hypertrophy in the affected extremity. The ability to provide safe anesthesia care for a patient with KTWS requires the practitioner to gain a full understanding of the syndrome and how it differs from similar syndromes. In developing a course for anesthesia administration in this unique patient population, it is critical for the anesthesia provider to become familiar with the regions of the body affected by KTWS, particularly in the lumbar spine, oral cavity, and airway, since this knowledge will ultimately guide the anesthetic plan.