Ganglioglioma is a rare tumor of the central nervous system, occuring in all age group but most commonly in children and young adult. We reported a 6-year-old girl with pineal ganglioglioma presenting progressive enlargement of both breasts and impairment of bilateral occulomotor function. A contrast CT scan revealed a pineal region tumor with heterogeneous contrast enhancement and cystic component at lower part of the tumor. In MRI, the solid part of the tumor showed mixed low and intermediate signal intensity in T1WI, which turned to high signal intensity in T2WI and terogeneous enhancement, mainly high intensity, after Gadolinium-DTPA injection in T1WI. The tumor was totally removed and pathology proved it a rare pineal ganglioglioma.