Astroblastoma is a rare primary brain neoplasm that accounts for 0.45-2.8% of brain gliomas. Intraventricular localization is extremely rare. The authors report a case of well-differentiated completely intraventricular astroblastoma in a 6-year-old girl and review the relevant literature. Their patient presented with a 5-week history of progressive nausea and vomiting. Magnetic resonance (MR) imaging revealed a large, well-demarcated, solid-cystic mass in the left temporooccipital ventricular horn. Macroscopic radical resection of the tumor was performed via the superior temporal sulcus. The postoperative course was uneventful and no adjuvant therapy was administered after surgery. No recurrence was detected at 9-months follow-up. Gross-total resection has the greatest impact on patient survival. In differentiated tumors, recurrence is usually local, and adjuvant therapy is recommended after repeated resection for the treatment of recurrence. In patients harboring anaplastic astroblastoma, gross-total resection and adjuvant therapy after the initial surgery seems to be the best choice. It is important to distinguish astroblastoma from ependymoma in clinical practice because of the differences in therapeutic approaches.