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Left ventricular (LV) myxoma is particularly rare in children and has not been reported in infants. A five-month-old baby presented with a myxoma arising from the anterior, lateral, and superior aspect of the LV, causing severe left ventricular outflow tract obstruction. The LV was accessed through the conal septum after opening the right ventricular outflow. The child had transient complete heart block in the postoperative period. There was no recurrence of tumor at the nine-month follow-up.