Rhabdomyomata are probably the most common tumors that occur very rarely during infancy. In this paper, we report the case of a 49-day-old infant who was diagnosed by echocardiography examination with left ventricle outflow tract (LVOT) obstruction caused by rhabdomyoma. The infant underwent surgical approach, and her mass was shaved. Finally, she was discharged from hospital in good general condition. Six-month follow-up after the operation did not show any obstruction.