We report the successful management of a 28-year-old parturient with an autosomal recessive limb-girdle muscular dystrophy with severe restrictive lung disease, who required non-invasive positive pressure ventilation in the third trimester of her pregnancy. At 37 weeks' gestation she had combined spinal-epidural anaesthesia for emergency caesarean section and had an uncomplicated intra- and postoperative course. The anaesthetic management is discussed and the literature briefly reviewed.