Spinal muscular atrophy in pregnancy is rare and poses multiple problems for the anesthesiologist. The effects of dexmedetomidine on a parturient with spinal muscular atrophy have not previously been reported. There are also no in vivo data on placental transfer of dexmedetomidine and its effects on a human neonate. We report the hemodynamic, respiratory and sedative effects of dexmedetomidine on a parturient and neonate when used for awake fiberoptic intubation before cesarean section. A 35-year-old, gravida 4 para 0 aborta 3, 41-kg parturient at 35 weeks of gestation with spinal muscular atrophy presented for cesarean section. Dexmedetomidine was administered intravenously, total dose 1.84 microg/kg over 38 minutes, followed by fiberoptic endotracheal intubation. Dexmedetomidine was then discontinued and general anesthesia was induced. The baby was delivered 68 minutes after the dexmedetomidine infusion was discontinued at which time blood samples were obtained for measurement of dexmedetomidine. During administration of dexmedetomidine, maternal heart rate, blood pressure and oxygen saturation remained stable. Apgar scores at 1 and 5 min were 6 and 8. The fetal concentration of dexmedetomidine (540 pg/mL) indicates significant placental transfer, but significant adverse neonatal effects were not observed. Dexmedetomidine alone provided adequate sedation for awake intubation without respiratory compromise in this patient.