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.