Rhabdomyoma is the most common fetal cardiac tumor accounting for 60–65% of all fetal tumors and up to 90% of cardiac tumors in pediatric population. Rhabdomyomas are benign lesions and the symptoms depend on the size and location of the rhabdomyomas. A single or multiple lesions are usually located within the left ventricle and projecting into the ventricular cavity or moving freely as a pedunculated mass. Intracavitary growth may cause ventricular outflow tract obstruction, valvular compromise, and disruption of intracardiac blood flows, leading to congestive heart failure. Rhabdomyomas >30 mm in diameter, regardless of number and location, are associated with postnatal arrhythmia. Cardiac rhabdomyoma is associated with tuberous sclerosis in about 50–90% of cases. Rhabdomyomas can be detected as early as the second trimester by echocardiography which is the primary diagnostic tool for the evaluation of cardiac tumors. They usually grow until 32 weeks gestation due to the transmission of maternal estrogens to the fetus, and then gradually regress. When cardiac rhabdomyoma is suspected on fetal echocardiography, it is important to check the kidneys and brain for tuberous sclerosis complex (TSC)-specific lesions. The prognosis depends on the number, size, and location of the tumors, as well as the presence or absence of associated anomalies. Rhabdomyomas, single or multiple, in the majority of cases, are not hemodynamically relevant and regress in infancy. Expectant management is the recommended approach, and neonatal management is always preferred when lung maturity is assured. Prenatal therapy is recommended only in case of severe fetal hemodynamic deterioration, hydrops, or uncontrollable arrhythmias.