A double-chambered right ventricle (DCRV) is a rare congenital heart defect. The clinical features of DCRV, especially in the pediatric population, have not been sufficiently elaborated. There are many unanswered questions regarding the surgical indications, surgical timing, natural history, and patient outcomes. This article will discuss the definition, pathophysiology, clinical features and treatment of DCRV. This systematic review showed a higher prevalence of symptomatic patients among children with DCRV with signs of right ventricular pressure overload and associated congenital heart defects. The spatial relation between ventricular septal defect and the anomalous muscle bundle might be a determinant of right ventricular obstruction. Symptomatic patients with an intra-right ventricular pressure gradient of >20 mmHg are indicated for a surgical repair. DCRV has a favourable postoperative course as no postoperative death was reported. Mechanisms of right ventricular obstruction warrant further elucidations based on larger patient population in the near future.