Background: Coarctation of the aorta (CoA) with aortic arch hypoplasia (AAH) is a relatively common congenital heart disease in clinical practice. Nonetheless, the corrective surgical technique for infants and children is a clinical problem that remains controversial. In this study, we sought to evaluate the surgical effects of aortic arch (AA) reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch for infants and young children with CoA and AAH. Methods: Between January 2009 and December 2015, a total of 22 infants and young children with CoA and AAH who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch were enrolled in this study. The median age of patients was 4.5 (Q1, Q3: 2.0, 14.0) months and the median body weight was 5.75 (Q1, Q3: 4.10, 9.38) kg. All patients were diagnosed with CoA and AAH, and concomitant cardiac anomalies were corrected in one stage. Perioperative and postoperative data were collected and analyzed using the paired sample t-test. Results: No perioperative deaths occurred. No residual obstruction was detected by echocardiography. The postoperative pressure difference across the repaired segment of CoA was 14.05 ± 4.26 mmHg (1 mmHg = 0.133 kPa), which was smaller than the preoperative pressure difference (48.30 ± 15.73 mmHg; t = -10.119, P < 0.001). The median follow-up time was 29.0 (Q1, Q3: 15.5, 57.3) months. There was no death during the follow-up period, and all patients experienced obvious clinical improvement. Only one child underwent subsequent aortic balloon angioplasty due to restenosis. Computed tomography angiography showed that the AA morphology was smooth, with no aortic aneurysm or angulation deformity. Conclusion: AA reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch could effectively correct CoA with AAH, and the rate of reintervention for restenosis is low.