A broad group of tumors can occur in the fourth ventricle. Medulloblastoma is the most common malignant tumor that occurs in the fourth ventricle. They account for one third of all childhood fourth ventricular tumors. Other types as Ependymomas and Astrocytomas take place in fourth ventricle. Clinical triad of increased intracranial tension- headache, vomiting and blurred vision is the main presenting features of fourth ventricular tumors due to obstructive hydrocephalus. Marked progress in radioimaging including CT and MRI had a great role in early diagnosis of fourth ventricular tumors. Some of neurosurgeons prefer to shunt the hydrocephalus before tumor removal but the majority prefer to deal with the tumor directly by total removal. Post–operative adjunctive treatment including radiotherapy and chemotherapy had improved the prognosis and long term survival of the patients.