<p>Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital disorder characterized by multiple venous malformations, most frequently affecting the skin and gastrointestinal tract, but occasionally involving the central nervous system and other visceral organs. Fatal outcomes are exceedingly rare and usually result from acute hemorrhage or airway compromise. This report describes a fatal case of BRBNS in a 31-year-old woman who developed uncontrollable bleeding from vascular malformations following an emergent tracheostomy performed for anaphylaxis secondary to lidocaine administration during dental treatment. Despite aggressive resuscitative measures, the patient succumbed to hemorrhagic shock. External examination revealed bluish subcutaneous lesions in the perioral region, periorbital ecchymosis, and multiple bluish, compressible subcutaneous vascular “blebs” on the right upper extremity, extending to the right hemithorax. Internally, multiple submucosal, hemorrhagic-appearing lesions were identified on the base of the tongue, hypopharynx, larynx, and aryepiglottic folds, with additional vascular malformations in the posterior mediastinum and right temporalis muscle. Histologic examination confirmed cavernous hemangiomas at the tracheostomy site. A review of the English-language literature revealed only a small number of fatal cases associated with BRBNS. This case highlights the potential for fatal airway hemorrhage in BRBNS, particularly during emergent procedures involving the upper airway. Awareness of possible laryngeal and tracheal involvement is crucial to avoid iatrogenic complications. Comprehensive autopsy remains essential to determine the cause of death, document lesion distribution, and distinguish disease-related hemorrhage from procedural injury.</p>