The muscular dystrophies are degenerative muscle diseases characterized by progressive muscle weakness. The vast majority of women suffering from muscle diseases develop a deterioration of symptoms during pregnancy. Cardiac and respiratory complications are observed in pregnant women with muscular dystrophy especially in the second and third trimester. The successful perioperative therapy of a 32-year-old pregnant tetraplegic woman with a severe limb-girdle type muscular dystrophy who underwent elective Caesarean section is reported. According to the literature epidural and spinal anesthesia are both possible for perioperative anesthetic management in women with limb-girdle dystrophies. Due to the excellent controllability of intrathecal hyperbaric bupivacaine it was decided to use spinal anesthesia and non-invasive positive pressure ventilation was planned in case of impairment of respiratory function. In summary limb-girdle muscular dysthrophies should be managed on an individual basis and spinal anesthesia can be safely used to avoid intubation.