show clearly that when patients with biallelic mutations in AKR1D1 develop liver disease, the liver disease improves with CDCA or CA treatment, but do all individuals with pathogenic mutations in AKR1D1 and a urine bile acid profile indicating that 3‐oxo‐Δ4 bile acids are the major cholanoids in the urine need treatment? Here, AKR1D1 is linked to liver disorder.