Carnitine palmitoyl transferase (CPT) type 2 deficiency is a disorder of mitochondrial fatty acid oxidation. In situations where energy stores are inadequate, such as may occur during labour women with CPT type 2 deficiency are at risk of rhabdomyolysis. There is limited experience in the management of women with this condition in labour. We report a case of successful labour management of a woman with CPT type 2 deficiency, together with a brief review of the published case reports and a discussion of the issues surrounding anaesthetic management.