Eight children with autosomal recessive polycystic kidney disease (ARPKD) a
nd recurrent bacteremia with enteric pathogens are described. Typical clini
cal features of bacterial cholangitis were absent, although in five patient
s histological and/or microbiological data indicated that the bacteremic ep
isodes originated in the biliary tree. Bacteremia with enteric pathogens or
recurrent culture-negative febrile illness in a child with ARPKD should ra
ise suspicion of cholangitis, even in the absence of typical clinical findi
ngs.