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Background and Aims Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy, affecting both sexes equally. It is characterised by progressive muscle weakness in the facial, shoulder girdle, and upper arm muscles. As the disease progresses, it may involve truncal and abdominal muscles, complicating the second stage of labor and often necessitating a caesarean section. Understanding the systemic involvement of FSHD is crucial for anaesthetists to safely navigate the peri-operative period. Regional anaesthesia is the preferred method, despite the risks of worsening and prolonged motor weakness. General anaesthesia is reserved for emergencies. Although malignant hyperthermia is not more common in FSHD, caution is advised. Pulmonary involvement, leading to restrictive disease and respiratory muscle weakness, poses significant risks for the parturient.

Methods A 25-year-old pregnant woman with a history of FSHD was admitted for a scheduled caesarean section. Given the risks and challenges associated with both general and regional anaesthesia, combined spinal epidural anaesthesia was chosen in this patient.

Results The caesarean section was performed under combined spinal epidural anaesthesia without any intraoperative complications. The patient‘s motor status recovered to pre-operative levels within the usual timespan, indicating a successful management strategy.

Conclusions Regional anaesthesia, particularly combined spinal epidural anaesthesia, is a viable and safe option for caesarean sections in patients with FSHD, despite the inherent risks of motor weakness and respiratory complications. This case highlights the importance of a tailored anaesthetic plan to address the unique challenges presented by parturients with FSHD, ensuring safety and effective pain management for both mother and child.