BACKGROUND: Pustular psoriasis (PP) is a systemic inflammatory disease that rarely occurs in children but adversely affects their quality of life. OBJECTIVE: This study aimed to evaluate the clinical characteristics, treatment, prognosis, and epidemiology of PP in pediatrics. METHODS: A single center retrospective study on 41 children diagnosed with PP was conducted in a university-affiliated referral hospital between January 2010 and May 2022. The demographics, clinical characteristics, treatment, and prognosis were evaluated in the descriptive analysis. To further investigate the effect of different acitretin-based treatments on the prognosis, 38 patients were included in the subgroup analysis. Patients who received acitretin alone were designated as the Acitretin group, while those who received acitretin combined with intravenous immunoglobulin, ciclosporin, or steroids (or any combination of these agents) were identified as the Combination group. RESULTS: A total of 41 patients were enrolled in the descriptive analysis. Of these, 65.8% (27/41) were male, and the mean age of onset was 3.2 ± 3.2 years. Among the patients, 63.4% (26/41) presented with pustules, and over half experienced fever and elevated C-reactive protein (CRP) levels. In the subgroup analysis, the CRP level was significantly higher in the Combination group (59.69 ± 43.74 versus 26.35 ± 19.67 mg/dL, P = 0.006), indicating that patients had more severe inflammatory conditions. Nevertheless, there was no significant difference in the remission rate between the Acitretin group and the Combination group. CONCLUSION: Pediatric PP is a disease that often occurs in the pre-school period and predominantly affects males. It is characterized by typical skin lesions and systemic inflammatory reactions. Common comorbidities include nutritional disorders such as malnutrition and anemia. Combination treatment based on acitretin is recommended for children with PP and severe inflammation.