Introduction <p>While rituximab has been approved for moderate-to-severe pemphigus vulgaris, data on pemphigus foliaceus (PF) remain limited because of the small number of patients included in therapeutic trials. However, current European guidelines recommend rituximab for the first-line treatment of patients with moderate-to-severe PF. This systematic review and meta-analysis aimed to evaluate the efficacy and safety of rituximab in PF.</p> Methods <p>This systematic review and meta-analysis was conducted according to PRISMA guidelines and registered in PROSPERO (CRD42023428459). Studies published in English that included ≥ 3 patients with PF treated with rituximab were identified through six databases up to May&#xa0;2025. Data on patient characteristics, rituximab regimens, clinical status at the end of follow-up, relapse rates, and severe adverse events (SAEs) were extracted.</p> Results <p>Twenty-four studies, published between 2012 and 2025, including 195 patients with PF, were analyzed. Mean age was 48.8 ± 20.8&#xa0;years, and median follow-up was 24.5&#xa0;months (interquartile range [IQR] 19–32.7). Rituximab was used as first-line therapy in 21% and as second-line therapy in 46.7% of patients (data not reported for 32.3% of patients), most frequently combined with oral corticosteroids (69.7%). The pooled complete remission (CR) rate was 75.0% (95% confidence interval (CI) 64.0–83.0; <i>I</i>² = 35.5%), with a median time to CR of 9&#xa0;months (IQR 3.8–14.0). No significant difference was found between the lymphoma- and the rheumatoid arthritis-type regimens (<i>p</i> = 0.80). The pooled relapse rate was 38.0% (95%&#xa0;CI 26.0–51.0; <i>I</i>² = 37.8%), occurring with a median time to relapse of 12.5&#xa0;months (IQR 10.25–24.5) after the first cycle of rituximab treatment. Twenty SAEs were reported, mainly infections (65%).</p> Conclusions <p>Rituximab appears to be a safe and effective treatment for PF. These findings support its use as first-line therapy in moderate-to-severe PF.</p>