A 31-year-old man was admitted to our hospital due to hydrocephalus with neurosarcoidosis. Ventriculo-peritoneal shunting was performed in the right lateral ventricle with intravenous methylprednisolone. Subsequently, after 4 months, additional ventriculo-peritoneal shunting in the left lateral ventricle was performed due to the enlarged left lateral ventricle and slit-like right lateral ventricle. After 6 months, he was re-admitted due to upward gaze palsy, and magnetic resonance image showed an isolated fourth ventricle with both the inlet and outlet of fourth ventricle obstructed by recurrent neurosarcoidosis. Owing to the difficulty of using an endoscope, we performed neuronavigator-guided ventriculo-peritoneal shunting via the left lateral transcerebellar approach for the treatment of the isolated fourth ventricle with intravenous methyl prednisolone. The patient was discharged with improved neurological status.