The use of radiofrequency ablation (RFA) for the management of supraventricular tachycardia (SVT) in infants and small children remains controversial. The aim of this study was to evaluate the safety and efficacy of RFA in critically ill small children (<1 year of age) with drug-resistant tachycardia accompanied by arrhythmogenic cardiomyopathy and heart failure. The study included 15 patients age 5.3 ± 3.7 months. Wolff-Parkinson-White syndrome and atrial tachycardia were detected in eight (53.3 %) and seven (46.7 %) of patients, respectively. Patients with structural heart pathology, including congenital heart diseases and laboratory-confirmed myocarditis, were excluded from the study. Indications for RFA included drag-refractory SVT accompanied by arrhythmogenic cardiomyopathy and heart failure. Unsuccessful ablation was observed in two 1-month-old patients who underwent successful ablation 3 months later. The follow-up period ranged from 0.5 to 8 years (average 3.9). Only one patient (6.7 %) had tachycardia recurrence 1 month after RFA. The short- and long-term RFA success rates were 86.7 and 93.3 %, respectively. The study did not show any procedure-related complications. Heart failure disappeared within 5-7 days. Complete normalization of heart chamber sizes was documented within 1 month after effective RFA. A three-dimensional CARTO system (Biosense Webster, Inc., USA) was used in three patients with body weight >7 kg. The use of the CARTO system resulted in a remarkable decrease of the fluoroscopy time without vascular injury or other procedure-related complications in all cases. Our study suggests that RFA may be considered the method of choice for SVT treatment in small children when drug therapy is ineffective and arrhythmogenic cardiomyopathy progresses.