Over the last 10 years we have seen 31 children with systemic lupus: 10 (32%) of these have died. The commonest primary determinant of mortality was uncontrolled disease activity (60%), possibly contributed to by late referrals leading to delays in diagnosis and the institution of therapy. Thromboembolic disease was responsible for two deaths and tuberculosis for one. The major contributory factors were nephritis in two patients and myocarditis, endocarditis, lupus pneumonia and CNS disease in one patient each. Concomitant infections, predominantly nosocomial, occurred in a significant proportion of patients (40%) but were only cofactors in mortality. Autopsies were done in three cases. These patterns of death are significantly different from those seen in the developed world, where disease activity has ceased to be an important factor owing to early recognition of cases. Avoiding late referrals and delays in diagnosis can reduce mortality in childhood-onset lupus.