Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial brain tumor occurring mostly before the age of 2 years. It has a good prognosis and total excision of the tumor is curative, necessitating no further treatment. An accurate pathologic diagnosis is crucial. Until now, <60 cases of this tumor type have been reported. Herein, we report a 3-month-old boy with intractable seizure who was found to have DIG after surgery.