Desmoplastic infantile ganglioglioma is an uncommon variety of ganglioglioma that shows evidence of glial andganglionic differentiation accompanied by an extreme desmoplastic reaction. A 16-month-old girl was ad-mitted witha six-day history of left hemiparesis. MR imaging demonstrated a large multiseptated cystic mass, with a solidportion, in the white matter of the right frontotemporoparietal lobe. After contrast injections, the solid portionwas clearly enhanced. The presence of desmoplastic infantile ganglioglioma was confirmed by surgical resection. Wedescribe the characteristic radiologic and pathologic features of desmoplastic infantile ganglioglioma, andinclude a a review of the literature.