Pre-emptive awake airway management under dexmedetomidine sedation in a parturient with spinal muscular atrophy type-2

•Pregnancy is generally considered contraindicated in spinal muscular atrophy.•With the advent of better care, more of these women are choosing to become pregnant.•This disorder complicates pregnancy due to lung disease and severe scoliosis.•Awake tracheal mature stoma creation was used in a woman with a known difficult airway. Historically, pregnancy in females with spinal muscular atrophy was contraindicated due to the great risk to the parturient, but with improved management and increased survival more patients are becoming pregnant. We describe the management of a pregnant patient with spinal muscular atrophy type-2, who had severe restrictive lung disease, extensive spinal fusion that precluded neuraxial anesthesia, and chronic respiratory failure on nocturnal Bilevel Positive Airway Pressure. Airway management was further complicated by limited mouth opening and cervical spine ankylosis.