Cobb syndrome describes the association of a spinal angioma and a cutaneous angioma such as nevus flammeus or angiokeratoma of the corresponding dermatome. A 23-year-old Korean female showed spiral cord angiomas and segmentally distributed nevus flammeus of corresponding dermatomes (Th5-Th2). Interestingly she had a lymphangioma circumscriptum in the same dermatomes. To the best of our knowledge, there is no report of this constellation. Therefore, we suggest that lymphangioma circumscriptum may be considered as a skin finding of Cobb syndrome.