In order to assess the systemic prognosis of children with leukaemic ocular involvement, 63 of 131 patients admitted to hospital with acute leukaemia were evaluated ophthalmically. A total of 28 of 63 showed ophthalmic involvement and were followed up for up to 84 months. Twenty seven of 28 patients (96.4%) died within 28 months after the onset of ocular involvement and within 83 months after the onset of leukaemia. The 5 year survival rate of patients with ophthalmic manifestations was 21.4% (6/28). This survival rate was significantly lower than that of those who lacked ophthalmic manifestations (16/35: 45.7%, p < 0.05). All of the patients with ophthalmic manifestations had either bone marrow relapse or central nervous system leukaemia. The prognosis was related to risk factors such as central nervous system leukaemia or bone marrow relapse in most cases.