An 11-year-old boy presented with a 4-month history of progressive holocranial headache, intermittent vomiting and visual blurring. Later, he began walking unsteadily, with progressive bilateral visual and hearing loss. He had had two episodes of abnormal tonic posturing the day prior to admission. On examination, the patient was drowsy and did not respond to commands. Imaging showed an enhancing midbrain mass with an exophytic component with hydrocephalus. Emergency ventriculoperitoneal shunt was performed, following which there was neurological improvement. Later he underwent a midline suboccipital craniectomy with vermian splitting and decompression of the exophytic component of the tumor from the midbrain. Biopsy of the tumor revealed a germinoma. Post-operatively, the patient received chemotherapy followed by radiotherapy. Primary midbrain germinoma is an extremely rare entity and to the best of our knowledge only two cases to date have been reported in the literature.