Background: Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes. Aim: To study quantitative liver functions in TS in detail with and without HRT. Design: Randomized crossover study with active treatment (HRT in TS and P-pill in controls) or no treatment. Subjects: Women with TS (nZ8, age 29.7G5.6 (meanGS.D.) years), verified by karyotype, and agematched controls (C; nZ8, age 27.3G4.9 years). Methods: We determined liver enzymes in blood, used the galactose elimination capacity to assess hepatocyte cytosol activity, plasma clearance of indocyanine green to assess excretory function, antipyrine clearance to estimate microsomal activity, and the functional hepatic nitrogen clearance (FHNC) to assess mitochondrial-cytosolic metabolic capacity for conversion of amino-nitrogen. Results: Liver enzymes were elevated in untreated TS and reduced by HRT. The hepatic capacities for conversion of galactose, indocyanine green, and antipyrine were normal and did not change by HRT.