Background - Intrahepatic cholestasis secondary to paucity of bile duct is an alteration of the anatomic integrity of the biliary tract. Can be defined only histologically and, clinically, two categories are recognized: syndromic and non-syndromic, where the prognosis is generally more severe. Aim - To evaluate the history, clinical and biochemical characteristics, etiology and improvement of children who have paucity of intrahepatic bile duct followed at tertiary center. Patients and Methods - Eleven children with paucity of intrahepatic bile duct, followed at the Pediatric Hepatology Service of the University Hospital, Campinas, SP, Brazil, were evaluated in the period from 1986 to 2001. Results - Among the patients, three presented the syndromic and eight the non-syndromic form (two with α-1-antitrypsin deficiency, one with lues, one secondary to sepsis, three with probable etiology by cytomegalovirus and one without a definite etiology). Referral ranged from 31 to 1185 days. Birth weights ranged from 1920 g to 3590 g. Most of the patients presented pale stools. The median bile duct/portal tract ratio was 0.14. The majority of the children presented a favorable follow-up, regardless of the form of presentation. Conclusion - Paucity of intrahepatic bile ducts should be considered in children with cholestasis and its differentiation from extrahepatic causes of neonatal cholestasis is important in order to avoid surgery. Diagnosis of non-syndromic form should not be regarded as unfavorable prognosis, as the evolution is probably related to the etiology in this form of presentation.413190192Alagille, D., Prolonged obstructive jaundice including calculous and noncalculous gallbladder conditions (1995) Pediatric Clinical Gastroenterology, pp. 636-671. , Roy CC, Silverman A, Allagille D, editors. St. Louis: MosbyBerezin, S., Beneck, D., Altman, P.E., Schwarz, S., Resolution of nonsyndromic paucity of intrahepatic bile ducts in infancy (1995) Dig Dis Sci, 40, pp. 82-85Colestase do recém-nascido e do lactente jovem (1995) Rev Paul Pediatr, 13, pp. 134-136Finegold, M.J., Carpenter, R.J., Obliterative cholangitis due to cytomegalovirus: A possible precursor of paucity of intrahepatic bile ducts (1982) Hum Pathol, 13, pp. 662-665Kage, M., Kosai, K.-I., Kojiro, M., Nakamura, Y., Fukuda, S., Infantile cholestasis due to cytomegalovirus infection of liver (1993) Arch Pathol Med, 117, pp. 942-944Koçak, N., Gurakan, F., Yüce, A., Çaglar, M., Kale, G., Gogus, S., Nonsyndromic paucity of interlobular bile ducts: Clinical and laboratory findings of 10 cases (1997) J Pediatr Gastroenterol Nutr, 24, pp. 44-48Krantz, I.D., Piccoli, D.A., Spinner, N.B., Alagille syndrome (1997) J Med Genet, 34, pp. 152-157Krantz, I.D., Piccoli, D.A., Spinner, N.B., Clinical and molecular genetics of Alagille syndrome (1999) Curr Opin Pediatr, 11, pp. 558-564Macasaet, F.F., Holley, K.F., Smith, T.F., Cytomegalovirus studies of autopsy tissue. II - Incidence of inclusion bodies and related pathologic data (1975) Am J Clin Pathol, 63, pp. 859-865Silveira, T.R., Pires, A.L.G., Icterícia colestática neonatal (1991) Gastroenterologia Pediátrica, pp. 465-487. , Penna FJ, Wehba J, Fagundes-Neto U, editores. Rio de Janeiro: MEDSISugiura, H., Hayashi, M., Koshida, R., Watanabe, R., Nakamura, Y., Ohta, G., Nonsyndromic paucity of intrahepatic bile ducts in congenital syphilis. A case report (1988) Acta Pathol Jpn, 38, pp. 1061-1068Tresoldi, A.T., Hepatometria em crianças: Valores de referência, para crianças de zero a 10 anos, de algumas medidas estimadas ao exame fisico (1987) J Pediatr (Rio de J), 66, pp. 13-17Valencia-Mayoral, P., Calva-Rodríguez, R., Ahumada-Maldonado, H., Hipoplasiade conductos biliares intrahepaticos. Estudo clinicopatologico (1989) Gac Med Mex, 125, pp. 145-149Witzleben, C.L., Bile duct paucity ("intrahepatic atresia") (1982) Perspect Pediatr Pathol, 7, pp. 185-201