Pruritus is a severe symptom in patients with cholestatic hepatobiliary disease; it can greatly reduce the quality of life. Cholestatic itching often peaks in the evening and early night. It mainly occurs on the palms of the hands and soles of the feet but can also occur more generalised. The pathogenesis of cholestatic pruritus has not yet been completely clarified. Possible contributors are bile salts, histamine, progesterone metabolites and opioids. A relationship between these elements and the intensity of the itch has not, however, been demonstrated. Autotaxin, an enzyme that produces lysophosphatidic acid, has recently been identified as a possible pruritogen caused by cholestasis. Treatment is aimed at eliminating pruritogens with bile acid sequestrants (cholestyramine), managing the metabolism of pruritogens (rifampicin), and influencing the perception of itch by the central nervous system with µ-opioid antagonists or SSRIs. In cases of unbearable, treatment-resistant itching, consideration may be given to experimental therapies such as UV light therapy or nasobiliary drainage.