Unilateral nevoid telangiectasia (UNT) is a rare congenital or acquired benign cutaneous vascular anomaly first identified by Blaschko in 1899. It is characterized by superficial telangiectasias with a reticular or linear pattern on one side of the body, often following a dermatomal distribution. We present the first case of UNT documented in Saudi Arabia in a 12-year-old girl.