A 73-year-old female patient who had severe neuropathic pain due to sciatic nerve hypertrophy with the Klippel-Trenaunay Syndrome has been presented. Localized hypertrophic neuropathy is in one region and characterized by concentric proliferation of Schwann cells around the axon. It is very rare in the absence of generalized hypertrophic neuropathy. Very little is known about the etiology and the course of this neuropathy. Klippel-Trenaunay-Syndrome (KTS) is a rare syndrome characterized by hemangioma, abnormalities of the venous and lymphatic systems, and limb enlargement due to soft tissue and bone hypertrophy.