Larsen syndrome is a rare genetic connective tissue disorder caused by mutations in the filamin B gene leading to a complex and heterogeneous presentation involving multiple organ systems. Mode of inheritance is both autosomal dominant and autosomal recessive (less common). The phenotypical presentations include cervical spine abnormalities and other skeletal system abnormalities involving the face, hands, and feet. Respiratory system involvement may manifest as laryngeal malacia and/or tracheaobronchomalacia. Cardiac abnormalities may exist. Due to multiple skeletal system abnormalities, patients with Larsen syndrome may require repeated surgical and nonsurgical interventions presenting unique perioperative anesthetic challenges.