Background: Fetal cardiac rhabdomyoma is a rare condition. Case: We report a case with cardiac mass discovered in utero by prenatal ultrasonography at 33 weeks of gestational age. An echogenic round-oval shape mass at the interventricular septum protrudes to left ventricle was observed. Results: After birth, the baby was followed up for 7 months with echocardiography, physical examination, and computerized tomography scan, to rule out anything related to tuberous sclerosis. The prognosis depends on the size, site, number of tumors, and coexisting congenital abnormalities. Management highly depends on the presence of outflow tract obstruction of the heart. However, some cases may regress after birth.
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