Abstract Introduction Glioblastoma in the pineal region is extremely rare with only a few cases reported in the literature. Case presentation A 68-year-old man presented with a sudden deterioration manifesting as a headache, vomiting and gait disturbance. A magnetic resonance imaging study revealed a heterogeneously ring-enhanced mass in the pineal region. The mass was subtotally removed through the occipital transtentorial approach, and diagnosed as a glioblastoma. Conclusion We discuss the clinical course, radiological findings and treatment strategies of pineal glioblastoma with a review of the relevant literature.