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We present a case of ganglioglioma in the cerebellar vermis. A 2-year-old boy presented with symptoms and signs of raised intracranial pressure and the physical examination revealed large head and developmental delay. Magnetic resonance image demonstrated a hypointense mass in the cerebellar vermis on T1-weighted image with heterogeneous enhancement after gadolinium administration, mimicking a malignant tumor. A gross total resection was performed and pathologic findings demonstrated a ganglioglioma. There was no evidence of recurrence of the tumor during 16 months follow up period. Key words:Ganglioglioma;Cerebellum.