Andersen-Tawil syndrome (ATS) is characterized by ventricular arrhythmias, hypokalemic periodic paralysis and developmental anomalies. It is caused by mutations in the KCNJ2 gene that encodes for the alpha-subunit of Kir2.1, a K(+) channel responsible for cardiac repolarization. Providing effective therapy to reduce arrhythmia burden and risk of sudden death is challenging, especially in the context of pregnancy and childbirth. We report a case of a pregnant 27-year-old woman with an R218W mutation in the C-terminal interaction domain of KCNJ2 causing ATS. Regular cardiac and obstetric assessments were performed for the duration of the pregnancy, which carried to term and delivered successfully with potassium replacement and intravenous beta blockade. ATS is a rare and potentially lethal condition in which there is considerable genetic and phenotypic heterogeneity. Effective management strategies are directed at reducing symptoms, arrhythmia burden and sudden cardiac death. This case illustrates the challenges and approach to management of patients with ATS who are pregnant and undergo childbirth.