Desmoplastic infantile gangliogliomas (DIGs) are rare tumors during infancy. They often occur as huge cystic tumors in the frontal and parietal lobes, with their solid component being adjacent to the leptomeningeal membrane. This report presents a patient with DIG and intractable epilepsy. Due to hesitation by the patient’s family in allowing surgery, this is the first time that the chronological changes in DIG have been observed on imaging studies. During the follow-up, the tumor changed from a pure solid tumor to a cystic one, which is a typical picture of DIG. Surgical pathology confirmed the diagnosis of DIG. However, for a patient with epilepsy, it is recommended that this condition should be treated immediately with surgery.