To clarify further the clinical correlates of antinuclear antibodies (ANA) in children with juvenile rheumatoid arthritis (JRA) this study compared the features of 60 ANA positive and 25 ANA negative children with JRA. ANA was more likely to be present in those with pauciarticular JRA than polyarticular JRA particularly if the ANA was of high titer. ANA positive subjects were more likely to have extraarticular manifestations, especially iridocyclitis. No significant differences were observed in onset ages, sex distribution, season of disease onset, family histories, or prognosis. There was no correlation between ANA titer and disease activity. Thus, while certain clinical features do correlate with ANA positivity in JRA, most clinical manifestations do not occur with distinctively different frequencies in the ANA negative and ANA positive groups.