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SUMMARY: Desmoplastic infantile tumors are rare supratentorial brain tumors that occur in pediatric patients. Desmoplastic infantile tumors are made up of 2 subtypes: desmoplastic infantile gangliogliomas and desmoplastic infantile astrocytomas. Desmoplastic infantile tumors are often identifiable on imaging on the basis of multiple characteristics. Nevertheless, pathologic analysis is required to confirm the diagnosis, particularly when the imaging features are atypical. Here, the radiology findings, surgical approach and subsequent management, and pathology of a desmoplastic infantile ganglioglioma are described.