Abstract Introduction Isolated H-type tracheoesophageal fistula has been distinguished in the group of congenital anomalies of the trachea and esophagus. It is a rare defect that causes diagnostic difficulties even for experienced clinicians. Material and Methods A retrospective analysis of the medical history of patients, who were operated in the Department of Pediatric Surgery and Urology in Wroclaw between 2009 and 2018 due to H-type tracheoesophageal fistula, was performed. Results The study group consists of 7 patients (4 boys and 3 girls). The diagnosis was made postnatally between 1 day of life and 4 months of age. The spectrum of symptoms was broad and varied from saturation disturbances to difficulties in food intake and recurrent pneumonia. In all patients, a contrast study was performed (the result of which was not always unambiguous). After diagnosis of the H-type fistula in bronchoscopy, the thoracoscopy was performed. All procedures were performed using 5 mm scope and 3 mm instruments. The pressure used during procedures was between 3 and 6 mmHg. All patients underwent right-sided thoracoscopy. To help with fistula identification in 3 patients, an urethral catheter was left in the fistula during the bronchoscopy. The size and appearance of fistulas were variable. Clips or sutures were used to close the fistulas. After surgery, the nasogastric tube was left in the esophagus for 7 days. After a control contrast study, the tube was removed and the full oral feeding was started. In 6 patients, the primary procedure turned out to be the final and no complications were observed in the postoperative course. In one patient in the postoperative course, the chylothorax was observed, the treatment of which required a rethoracoscopy with successful local tissue electrocoagulation. Conclusions The congenital isolated tracheoesphageal fistula is a rare disorder. It causes unspecific clinical symptoms, therefore the diagnosis is often difficult. The thoracoscopic access in the hands of skilled surgeon is a safe method and should be the treatment of choice.