A 42-year-old male was admitted with nasal bleeding. On admission he showed no abnormal neurological sign. A diagnosis of acute lymphoblastic leukemia (ALL) (L1) was made, and modified L-10M protocol was performed. During consolidation chemotherapy, the bone marrow was in remission but he showed left facial palsy. Four days after the onset of left facial palsy, the patient developed bilateral facial palsy followed by left oculomotor and abducens nerve palsy. At this time, bilateral ocular fundus showed papilloedema, exudate, bleeding, Roth's spot and leukemic infiltration to the anterior segment of the eye. Systemic chemotherapy with intrathecal injection of anti-leukemic drugs and whole brain irradiation was partially successful against cranial nervous system (CNS) complaints, but the patient relapsed. Seven month later hypopyon and secondary glaucoma developed and trabeculectomy was performed. The bone marrow revealed an increase of leukemic blasts. Chemotherapy consisting of various drugs was not effective, and he died of pneumonia. In this case, although intrathecal chemotherapy and whole brain irradiation were attempted, leukemia evolved CNS, retina and uvea despite the fact that the bone marrow was in complete remission, allowing various cranial nerve signs and ocular manifestations to occur.