This paper describes the incidence, diagnostic clinical manifestations and severity of juvenile systemic lupus erythematosus (jSLE) in a cohort of New Zealand Māori and Pacific Island children compared to European children. A review of charts for 32 children with jSLE seen by the Starship paediatric rheumatology and/or renal services between January 2000 and November 2010 revealed an annual incidence of 0.52/100,000 per year. The incidence of jSLE was higher among Māori and Pacific (0.67/100,000 per year; p=0.06) and significantly higher among Asian children (1.17/100,000 per year; p=0.01) than among European children (0.31/100,000 per year). At presentation, lupus nephritis was diagnosed significantly more often in Māori and Pacific children compared with European children (80% vs 40%; p=0.09, as was severe (WHO class 4 or 5) renal lesions (60% vs 40%, p=0.43). Similarly, at any time during the study, lupus nephritis (100% vs 40%; p=0.001) and severe (WHO class 4 or 5) renal lesions (73.3% vs 40%; p=0.12) were more frequent among Māori and Pacific compared with European children. In retrospective analyses of British Isles Lupus Assessment Group (BILAG) scores, severe “Category A” disease occurred more often in Māori and Pacific children compared with European children (56.8% vs 22.7%; p=0.17) and was predominantly renal in nature (73.3% vs 40%; p=0.12).