A 49-year-old man with liver disease developed a unilateral telangiectatic lesion over the left trigeminal and left C3, C4, C5, and T1 dermatomal areas. This case fulfills the criteria for unilateral nevoid telangiectatic syndrome (UNTS), and as such represents the 11th reported case of UNTS and the first case, to our knowledge, of visceral involvement associated with a similar vascular malformation. A hemorrhagic tendency is present in this syndrome when there is gastrointestinal involvement.