Background: Ganglioglioma is a lesion rarely encountered in the pineal region. Only six cases have been published to our knowledge.
Methods and results: We present the case of a 38-year-old male suffering from a pineal ganglioglioma that was totally removed by surgery. Histological diagnosis was made by light microscopy using immunostaining for synaptophysin. We review the few radiological and pathological data available in the literature concerning this very rare lesion for which surgery alone provides excellent results.
Conclusion: The possibility of long term remission obtained by surgery alone and the lack of specificity of clinical and radiological features of ganglioglioma are additional reasons for an aggressive surgical management of pineal tumors when tumor markers and cytopathological examination of the cerebrospinal fluid are not contributive.