We reported two patients with neurocutaneous angiomatosis. Patient 1, a 36-year-old woman of Cobb syndrome had cutaneous portwine angiomas in the high back and spinal arteriovenous malformations at the level of Th4-11. She had also atrophy and livedo reticularis presenting during standing position in the left lower extremity. Patient 2, a 47-year-old man of Klippel-Trenaunay-Weber syndrome had varices in the bilateral lower extremities, cutaneous portwine angiomas in the high back and bilateral lower extremities, and hypertrophy of the bilateral legs. Neuroradiological examination revealed abnormal vessels in the spinal canal at the level of C5 and Th4. These syndromes may have no essential difference because of the presence of neural and cutaneous angiomas at the corresponding level, venous system disorders and trophic changes (hypertrophy/atrophy).