Gliofibroma is a rare tumor with biphasic morphology. We report a case of a 3-month-old girl presenting with increasing head circumference. CT and MRI showed the presence of a large heterogeneous, nonenhancing tumor filling the atria and bodies of the lateral ventricles with caudal extension into the third ventricle. Hydrocephalus was noted. A biopsy was performed and pathology showed the presence of glial and collagenous tissue consistent with the diagnosis of gliofibroma. However, no mitosis or necrosis was seen. Immunohistochemistry was positive for glial fibrillary acid protein, Masson trichrome and reticulin. The patient was followed up for a period of 10 years. Recent MRI showed stable appearance of the tumor and clinically the patient is neurologically intact with normal cognitive development. Our case report has the longest follow-up so far reported in the literature, of 10 years. As evident from our case report, we recommend a conservative treatment approach for gliofibroma with benign histology.

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