Unilateral nevoid telangiectasia (UNT) is characterized by the dermatomal distribution of telangiectasia, which are distributed unilaterally and frequently found in C3-C4 or the trigeminal dermatomal areas. It may be congenital or acquired and has a 2:1 female: male ratio. Several theories where developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in estrogen levels. Its incidence is unknown, although it's probably subdiagnosed and more frequent than previously recognized. We present two pediatric cases of UNT followed at our instution during the past year.