Glutaric aciduria type 1 (GA-1) presents unique challenges for anaesthetists. This case report discusses anaesthesia management in a pregnant woman with GA-1 undergoing cesarean delivery. Based on a cautious consideration of potential complications, combined spinal-epidural anaesthesia was preferred in this case. Maintenance of normoglycemia, normothermia, low-protein diet, carnitine supplementation, and proper hydration were prioritized. A healthy baby was delivered without complications. This case underscores the importance of comprehensive preoperative assessment and individualized anaesthesia strategies for achieving optimal outcomes in pregnant patients with GA-1. The cautious management of anaesthesia-related risks is important to ensure patient safety and decrease stress responses. Neuraxial anaesthesia and analgesia may be advantageous in specific cases.