The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) becomes the systemic ventricle pumping against systemic afterload. It also has to adapt to an initially increased volume load followed by a decrease in volume load after Fontan completion. Anatomical HLHS subtype, therapeutic strategy, tricuspid valve regurgitation, recoarctation, and genetics influence RV size and function. The resulting remodeling process can be maladaptive and lead to ventricular systolic and diastolic dysfunction. While systolic dysfunction is a strong predictor for mortality before Fontan, there is increasing evidence for the impact of progressive diastolic dysfunction after Fontan. This comprehensive review summarizes the (recent) empirical observations that increased understanding of RV remodeling and function in HLHS. It aims at clinicians and researchers wishing to increase their understanding of the physiology of this disease. It highlights the potential for future scientific work on the assessment and preservation of myocardial health throughout the palliation.