Medulloepithelioma is a rare tumour with a very poor prognosis. The most frequent site in the central nervous system is the cerebral hemispheres. Appearances on MRI are very variable, possibly reflecting the divergent forms of differentiation seen at histological examination. We report a rare case of sellar and suprasellar medulloepithelioma in a 2-year-old boy and discuss the imaging, differential diagnosis and pathologic findings.