Benign recurrent intrahepatic cholestasis is characterized by attacks of jaundice with obstructive features recurring over a number of years, and without evolution towards biliary cirrhosis. The etiology is still obscure. The 1st episode of jaundice occurred before the age of 30 in all but 1 and before the age of 20 in 13 patients. Episodes of jaundice are invariably accompanied by severe itching and vary often by fatigue and weight loss. Tests of hepatic function showed cholestatic features. Serum cholesterol levels are not strikingly elevated. An operative cholangiogram for the demonstration of a normal biliary tree is essential for the diagnosis. Treatment is merely suportive and steroid therapy shows no consistent therapeutic effect. Temporary relief from jaundice may be obtained with surgical drainage. Cholestyramine has also been suggested. Light microscopy study showed only cholestasis and, in one case, a slight portal fibrosis. Electron microscopy disclosed, besides altered or absent microvilli of the bile canaliculi, the presence in the cytoplasm of the hepatic cells of moderately electron dense vesicles surrounded by a single or double membrane. This last finding may be specific for this disease. These electron microscopy findings persisted during the anicteric phase of the disease.