Congenital cardiac fibromas are very rare and prenatal diagnosis has been reported in just a few cases. We describe a four-month-old infant presenting a symptomatic giant right ventricular fibroma discovered during prenatal scanning at 33 weeks of gestation, which was confirmed after delivery on echocardiogram and cardiac magnetic resonance imaging. Due to progressive hemodynamic deterioration, partial surgical resection was performed and the patient recovered uneventfully. We report the successful management during early infancy of a giant cardiac fibroma prenatally diagnosed.