A 17-year-old male presented with morning headache and double vision. Neuroimaging revealed a lobulated enhanced mass lesion with a blurred margin and remarkable peritumoral edema, and high uptake of methionine. The gray, soft, well-circumscribed mass was grossly totally resected. Histological examination showed the tumor cells were well differentiated with the perivascular pseudorosette pattern with broad, non-tapering processes radiating towards a central vessel without anaplastic features such as necrosis and endothelial proliferation. The histological diagnosis was low-grade astroblastoma. Follow-up magnetic resonance imaging demonstrated local recurrence 5 months later. Second surgery was followed by adjuvant radiotherapy and combination chemotherapy. Histological examination disclosed wide invasion by tumor cells into the subpial and perivascular space of the surrounding brain tissue. Follow-up magnetic resonance imaging demonstrated further recurrence around the tumor cavity. Surgical removal followed by six courses of combination chemotherapy (ifosfamide, cisplatin, and etoposide) resulted in complete remission of the tumor. Although gross total resection of astroblastoma usually results in long-term survival, some of these yet unfamiliar tumors may develop a more malignant character.