To examine the relationship between physical appearance concern and psychological distress in female adolescent patients with systemic lupus erythematosus (SLE) was the aim of this study. A total of 84 adolescent SLE female patients and 80 age-matching healthy adolescents were evaluated for levels of appearance concern and a range of illness-specific measures to determine how these demographic and clinical variables were related to the dependent variable psychological distress. The Systemic Lupus Erythematosus Disease Activity Index was used to assess disease activity. Assessment of depression was conducted through the children depression inventory (CDI). Appearance concern was measured using the Self-Perception Profile for Children. The total CDI was 18.5 ± 4.3, indicating that these patients had more depressive symptoms, comparing with age- and sex-matched healthy controls. Furthermore, a total of 32 (38.1 %) patients had CDI larger than 19 points, indicating that they have relatively higher risk in developing depression. The CDI in control group was significantly lower than that in the SLE group (11.4 ± 1.7 vs. 18.5 ± 4.3, t = 9.93, p < 0.05). Using correlation and multiple regression analyses, we found that both appearance concern and age were predictive of depression in patients with SLE, and the former was highly correlated. This indicates that appearance concern may be associated with depression in female adolescent SLE patients. The results suggest that appearance concern is strongly associated with depression in female adolescent patients with SLE and should be routinely assessed.