Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare systemic vascular malformation-related disorder that primarily affects the skin, gastrointestinal tract, and central nervous system. Approximately 55.9% of patients have lesions involving the oral and maxillofacial region. Due to the potential risks of gastrointestinal hemorrhage and intracerebral hemorrhage, it is crucial for dental practitioners to promptly identify and refer such cases to relevant departments to improve patient prognosis and enhance quality of life. This paper reports two cases of BRBNS associated with maxillofacial vascular malformations treated at the Oral and Maxillofacial Surgery Department of Henan Provincial People’s Hospital. In conjunction with a literature review, this study discusses the clinical features, diagnosis, and treatment strategies of BRBNS, aiming to raise awareness among dental practitioners to avoid missed or incorrect diagnoses.