Objective -The aim of this case report is to present the results of surgical treatment of a patient with the pineal germinoma. Case report -A 12-year-old patient presented with two months history of headache, nausea, and vomiting, following by neuro-ophthalmologic disturbances (Parinaud syndrome). Computed tomography of the head showed a 3 cm mass in the pineal region, followed by obstructive hydrocephalus, and magnetic resonance imaging showed a pineal gland lesion with heterogeneous enhancement on contrast studies, with surrounding peritumoral edema, suggesting pineal germinoma. The patient underwent surgery to place a ventricular-peritoneal shunt, and second surgery using the occipital interhemispheric transtentorial approach to remove the tumor totally. Conclusion -Pineal germinomas that compress adjacent structures result in a typical clinical syndrome with endocrine malfunction, hydrocephalus or neuro-ophthalmological disturbances. A multimodality approach, including chemotherapy, radiotherapy and surgery, can offer excellent chances of free survival, and even cure.