Background: Beals syndrome, or congenital contractural arachnodactyly (CCA), is a rare autosomal dominant connective tissue disorder caused by pathogenic variants of FBN2. The association with diffuse vascular malformations is exceptional. Airway management during anesthesia is challenging due to joint contractures and restricted cervical mobility. Case presentation: We report the case of an 11-year-old girl with genetically confirmed Beals syndrome who underwent resection of multiple angiomatous lesions of the limbs. Preoperative evaluation revealed a marfanoid habitus, arachnodactyly, joint contractures, and limited neck extension. Cardiac function was normal. A difficult airway was anticipated, and mult iple devices were prepared (videolaryngoscope, flexible bronchoscope, supraglottic airways). Management and Outcome: Induction was performed with preservation of spontaneous ventilation. Endotracheal intubation was successfully achieved using a videolaryngoscope. Surgical dissection proceeded cautiously due to tissue fragility, and no major hemorrhage occurred. The patient was extubated safely in the recovery unit, and her postoperative course was uneventful. Conclusion: This case emphasizes the importance of structured anesthetic planning for airway management and vigilant perioperative monitoring to prevent complications in children with Beals syndrome.
