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Pregnancy with limb-girdle muscular dystrophy (LGMD) R1 calpain3-related is extremely rare, and its prenatal management is unclear. A primigravida in her late 30s with LGMD R1 calpain3-related was referred to our hospital during the first trimester. Rehabilitation was implemented in the second trimester owing to worsening muscle weakness in the lower extremities. Cardiopulmonary function was determined to be tolerable for vaginal delivery. Vaginal delivery with epidural anaesthesia was achieved to prevent irreversible muscle damage caused by straining or caesarean section. We performed a multidisciplinary simulation in the third trimester for safe delivery to confirm the patient’s lithotomy position and number of staff members required. At 38 weeks of gestation, labour was induced with epidural anaesthesia and instrumental delivery after a 4 hours second stage. A male neonate weighing 2848 g, with an umbilical cord artery pH of 7.27 and Apgar scores of 8 and 9 was delivered. With continuous postpartum rehabilitation, the muscle strength in the mother’s lower extremities recovered to the same level as that before pregnancy over 6 months. Her creatine kinase level was abnormally high, but similar to that observed prenatally.