There is a concern that percutaneous transvenous mitral commissurotomy (PTMC) performed during acute rheumatic fever (ARF) may result in adverse outcomes. We performed a retrospective study at a tertiary care center. Ten patients (ages 8-12 years) with severe symptomatic mitral stenosis and ARF who underwent PTMC (ARF group) were compared with 10 controls with similar mitral valve area (MVA). The procedure was successful in all patients. The median MVA increased from 0.7 to 1.5 cm2 following PTMC in the ARF group and from 0.7 to 1.8 cm2 in the control group [p = not significant (NS)]. The median MVA was 1.2 cm2 in the study group compared to 1.6 cm2 in the control group (p = 0.02) over a mean follow-up of 17.5 +/- 11.7 months. Echocardiographic restenosis occurred in 4 patients (40%) in the ARF group compared to 1 patient (10%) in the control group (p = NS). The improvement in the New York Heart Association functional class was maintained in 8 patients of the ARF group and 9 patients of the control group. PTMC can be performed in children with ARF without an increase in procedural complications. However, the restenosis rates appear to be higher.