Gangliogliomas are rare low-grade tumours composed of neoplastic glial and neuronal cell elements. Although they may arise within any part of the neuraxis, involvement of the brainstem is rarely encountered. The distinct nature of gangliogliomas means that they have a comparatively better prognosis than other tumours in this location following subtotal resection. We present the case of an infant with a pontomedullary ganglioglioma and discuss the relevant literature.