Congenital disorders of neuromuscular transmission are commonly referred to as congenital myasthenia gravis because of their clinical similarity to the immune-mediated disease. Differentiation between the immune-mediated and congenital forms of the disease is important, because therapy established for the former may not be appropriate for patients with the latter presentation. The course of this rare neuromuscular disorder during pregnancy and its influence on anaesthesia remain largely unknown. We report on the case of a 32-year-old parturient suffering from congenital myasthenia gravis scheduled for elective caesarean section. The perioperative management of this patient who underwent the operation under spinal anaesthesia was reviewed. The effects of anaesthetic agents and techniques on the course of congenital myasthenic patients may need further review in the light of latest findings in the electrophysiology, genetic and therapeutic studies of this syndrome.