Background:Reports concerning nonsyndromic paucity of the interlobular bile ducts are not common.Methods:The clinical, biochemical, and histological features of ten such children were described.Results:All presented with jaundice, starting in the first month in seven and in the fourth, seventeenth, and thirtieth month in the others. Acholic stools were present intermittently in seven and persistently in three patients. Pruritus was a prominent symptom in five. Liver function tests were abnormal in all but one. Liver biopsies were performed at ages of 20 days to 3 years (median 5 months). In addition to a paucity of interlobular bile ducts, histology revealed intracellular cholestasis in all, portal fibrosis in four, and regenerative nodules in two patients. Complications of fat-soluble vitamin deficiency occurred in seven. Therapy consisted of supplementation of those vitamins and administration of cholestyramine, phenobarbital, prednisolone, or ursodeoxycholic acid. While one child had a successful orthotopic liver transplantation, three died. Consanguinity rate was 80% among the parents, and five of the patients had siblings with similar symptoms.Conclusions:Prognosis of these patients is variable. Differentiation from other forms of cholestasis is important especially to avoid surgery.