Ganglioglioma are tumors presenting neoplastic glial cells and nerve cells, very rarely found in the pineal region. Only eight cases have been previously published in the literature. We present the case of a 14 years-old male patient with a ganglioglioma of the pineal region. The patient was treated surgically by a suboccipital transtentorial approach with complete removal. Histopathologic specimens with immunostaining revealed a ganglioglioma grade I. We conclude that these tumors are rare and should be treated surgically aiming total remove. If it is not possible or in case of reocurrence the patient should be followed and radiotherapy could be considered.