A 9-year-old male child had a IV ventricular medulloepithelioma of classical histology, showing tubulopapillary and undifferentiated areas. The unusual feature, however, was the presence of melanin pigmentation in the cells, which was further confirmed by electron microscopy. So far 28 cases of medulloepithelioma have been reported in the English literature. However, none of them showed melanin pigmentation. To the best of our knowledge this is the first case of pigmented medulloepithelioma in the English literature.