ABSTRACT
A case of a symptomatic spinal extradural meningeal cyst (SEMC) in Klippel-Trenaunay syndrome (KTS) is introduced. A 38-year-old woman presented with right L2 radiculopathy. She underwent operations for varicose veins in both her lower extremities. She had port-wine nevi on her trunk and extremities. The edematous change in both legs had waxed and waned. Magnetic resonance imaging showed an 11.8x13 mm extradural meningeal cyst growing through the intervertebral foramen in L2-3. Multiple meningeal cysts were located in the dorsal aspect of the spinal cord from T3 to T10. A 5.8x6.2 mm cyst was also found in left pleural cavity. The extradural meningeal cyst was completely excised and the preoperative symptom was improved. KTS is a congenital disorder due to a mesodermal abnormality, which may predispose the dura to weakness. The SEMC may occur through the dural defect or weakened point.