Objectives <p>Cirrhotic cardiomyopathy (CCM) is an under recognized entity in children. The present study aimed to explore the prevalence and natural history of CCM and its impact on outcomes among children with biliary atresia (BA) and genetic intrahepatic cholestasis (GIC).</p> Methods <p>This was a prospective observational study. All children aged 0–18&#xa0;years and diagnosed with either BA or GIC were included. Relevant clinical and biochemical parameters were recorded and transthoracic echocardiography was done. Left ventricular mass index (LVMI) ≥ 95&#xa0;g/m^2.7 and/or relative wall thickness of left ventricle ≥ 0.42 was used for defining CCM.</p> Results <p>Eighty nine children were included in the study, 64 with BA and 25 with GIC. Prevalence of CCM in children with BA and GIC were 53.13% and 48% respectively. LVMI (g/m^2.7) was significantly higher in children with unsuccessful KPE (p = 0.05 and p = 0.024) and unoperated BA (p &lt; 0.001 and p = 0.002) compared to those with successful KPE and GIC. Serum bile acid levels were significantly (p = 0.026) higher among children with CCM. CCM was associated with a lower native liver survival (NLS) (p = 0.0039) and overall survival (OS) (p = 0.006) at 12&#xa0;months. There were no significant associations between presence of CCM and post-LT mortality, time to extubation, duration of inotrope requirement or length of ICU stay. 80% children with CCM at baseline had resolution of CCM within 6&#xa0;months post-LT.</p> Conclusions <p>CCM is prevalent in almost half of patients with BA and GIC and is associated with decreased NLS and OS. Serum bile acids were significantly higher in children with CCM.</p>