The medulloepithelioma is a tumor too little frequent in adults, meeting described in literature only a group of ten of cases. We presented the case of patient submitted to enucleation of its straight eye by suspicion of melanoma uveal. The biopsy informed neoplasia of neural lineage of biological uncertain behavior, and the study immunohistochemistry informed neoplasia neuroendocrina of low grade. Study of systemic extension proved to be negative. The patient loses controls for 6 years, returning to consult for a great orbital right occupation, treated with orbital exenteration. The biopsy informed neoplasia malignant of neural lineage, and the histochemistry informed the pigmented malignant ocular medulloepithelioma with extension to the orbit. The patient evolved with the lesion's signs of complete remission, however a year and six months after begin with an increase of rapidly progressive volume, occupying all of the orbital region. Oncologic committee indicate study of systemic extension with LCR's cytology, TAC of thorax, abdomen and pelvis that proved to be normal; the osseous cintigrama evidenced activity osteoblastic pathological in facial region, and MRI of brain evidenced invasion intra and extra-cranial, with occupation of the paranasal sinus. The patient enters to the program of palliative cares and relief of the pain, receiving palliative radiation therapy, with partial remission of size tumoral and symptomatology.