Maternal anti-SSA antibodies are common, existing in up to 2 % of the general population. Fetuses exposed to these antibodies are at risk for both cardiac and noncardiac complications. The cardiac complications include arrhythmias, structural disease, and cardiomyopathy. Although rare, the cardiac disease associated with these antibodies is permanent and severe. Current fetal echocardiographic screening tools are nonspecific. The type and frequency of screening needed is controversial. Although promising transplacental treatment strategies exist, prospective randomized studies are lacking. Dexamethasone, the medication used most frequently, imposes significant risks to both mother and fetus. This report presents a discussion of the at-risk population, the spectrum of fetal cardiac disease associated with maternal anti-SSA antibodies, the current fetal echocardiographic screening tools, the therapeutic options, and the management and delivery planning strategies. With appropriate prenatal follow-up, assessment, and delivery planning, even high-risk fetuses can be delivered safely and managed effectively.