To describe the peripartum management of a patient with Isaacs' syndrome with specific reference to the anaesthetic implications of the disease process. Associated medical problems included obesity, pregnancy induced hypertension and a difficult airway.This 30-yr-old gravida V para 0 woman presented to the anaesthesia consultation clinic at 37-wk gestation to discuss pain relief options for labour and delivery. She had a history of Isaacs' syndrome (a peripheral motor neuron disorder), congenital heart disease (ASD and VSD), treated Hashimotos thyroiditis, obesity and a family history of haemachromatosis. On the day of consultation, she was hypertensive and peripheral oedema was noted. Her urine showed trace protein. Four days later, she presented to the labour suite and her cervix was 9 cm dilated. An epidural anaesthetic was given without difficulty and she had an uneventful labour and delivery course. There were no subsequent neurological complications.Isaacs' syndrome is an extremely rare peripheral motor neuron disorder. This patient was successfully managed with epidural analgesia for labour and delivered a healthy child with no congenital anomalies.