Three siblings with congenital dilatation of the intrahepatic bile ducts (Caroli's disease) are presented. Bile duct pathology was associated with congenital hepatic fibrosis and polycystic renal disease in all three patients. On color Doppler imaging (CD imaging), multiple small color Doppler signals were observed in the vascular radicles within the dilated bile ducts or in the center of the lumen apart from the vascular radicles, as well as in other well-known sonographic findings such as bile duct dilatations and bilary calculi. Doppler frequency spectral analysis confirmed all these color signals as arterial in origin in all patients, revealing pulsatile wave patterns. In spite of the fact that portal venous radicles have been well described on conventional sonograms or computed tomography (CT), continuous wave patterns of venous flow on spectral analysis were not detected in all patients. Identification of such less emphasized arterial flow may add another clue in the diagnosis and pathogenesis of this rare disease entity. In conclusion, color Doppler signals of arterial wave pattern within the dilated bile ducts are another helpful diagnostic criteria in previously reported sonographic findings, and these color signals are easily depicted on sonograms with color mapping.