Desmoplastic infantile ganglioglioma (DIG) has a favorable prognosis and is classified as a benign infantile brain tumor. The DIG is more common in children under 2 years of age than in other age groups. This report introduces a 5.5 month-old infant who was referred with infantile spasms and diagnosed with a brain tumor. Brain magnetic resonance imaging showed a large heterogeneous mass in the right hemisphere with shifting to the other side. The patient underwent surgery. The extra-axial mass was completely resected, and the diagnosis of DIG grade I was confirmed by pathology. After one year, patient development was normal, and the seizures did not recur. In addition, the general condition was good. With a brief review and search in the literature, 13 case reports were identified 9 of which were male cases. The mean age of initial manifestation to final tumor diagnosis was 4 months. Out of 13 patients, 8 cases were reported with the mass origin in the right hemisphere. The most commonly observed tumors were glioma (n=4) and hypothalamic hamartoma (n=3). Except for three patients who died, the remaining had a complete recovery after tumor removal with a seizure-free interval at follow-up.
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