The aim of the study was to study the case of a three-year-old girl with estrig-Weber syndrome without facial nevus. The study came to a number of conclusions, the most important of which is that the distinctive feature of SWS syndrome is cutaneous-facial venous dilatation in the distribution of ocular branches V1 and upper jaws V2 of the trigeminal nerve and they are present from birth; therefore, the syndrome can be detected early unlike capillary hemangiomas, which can only be detected with age.