Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous system vascular malformations. A 52-year-old woman in whom intracranial vascular malformations had been diagnosed earlier presented for evaluation. At birth, several blue nevi had been noted on her tongue, lips, and neck. Cerebral angiography demonstrated an extensive network of developmental venous anomalies and a left anterior sinus pericranii. The literature on BRBNS and developmental venous anomalies is reviewed.