Fetal cardiac rhabdomyoma is the most common cardiac tumor in fetuses. However, this benign tumor can cause hemodynamic repercussions and intrauterine fetal mortality. The present study reports a case of rare fetal cardiac rhabdomyoma located in the right atrium, accompanied by premature restriction of the foramen ovale and moderate pericardial effusion, as determined by tomographic ultrasound imaging (TUI). Fetal mortality subsequently occurred late in the second trimester of pregnancy and the diagnosis was confirmed by pathology. The present study discusses the occurrence and diagnosis of this rare abnormality. TUI mode with spatio-temporal image correlation offline imaging provides the physician with clear views of abnormal intracardiac structures in the beating heart. With improvements in sonographic technology, the diagnosis of fetal cardiac rhabdomyoma may be easier and more accurate in the clinical arena.