We report a patient with severe obstructive sleep apnea (OSA) associated with a unique combination of syringobulbia-myelia, Chiari malformation type I (CM), absent hypoxic ventilatory drive, vocal cord paralysis, post-menopausal status, obesity, and acute respiratory failure necessitating mechanical ventilation. The remote onset of OSA five years after surgery underscores the need for long-term follow-up of patients with syringobulbia-myelia and CM and the importance of addressing multiple interacting neurologic, metabolic, and mechanical predispositions to sleep-disordered breathing.