The farnesoid X receptor (FXR) is a member of the nuclear family of receptors and has emerged as a key player in the control of numerous metabolic pathways. Bile acids and their intermediates activate FXR, which thus serves as a sensor of bile acid levels. Along with retinoid X receptor (RXR), FXR plays a crucial role in linking bile acid regulation with lipoprotein, lipid and glucose metabolism. The concentration of bile acids and their intermediates can be elevated in conditions like cholestasis or inborn metabolic disorders. The metabolic syndrome is characterized by insulin resistance and glucose tolerance, abdominal obesity, hypertension, low high-density lipoproteins (HDL) and hypertriglyceridemia. As a modulator of bile, lipid and glucose homeostasis, FXR is an interesting pharmacological target for therapeutic applications.