Unilateral nevoid telangiectasia is characterized by unilateral occurrence of telangiectases assigned to certain dermatomes. Histologically, numerous thin-walled dilated vessels are seen in the upper and mid dermis without endothelial cell proliferation. Although it may be congenital or aquired, only acquired forms have been frequently reported in association with physiologic states of estrogen increase or estrogenized pathologic states, such as chronic hepatic disease in alcoholism. We report a case of congenital unilateral nevoid telangiectasia with extensive involvement of C4-T2, L1-S3 dermatomes in a 3-year-old girl.