Hepatocyte-specific magnetic resonance imaging (MRI) contrast agents are commonly used to depict anatomic hepatobiliary lesions and are also useful in characterizing the kinetics of hepatocyte uptake and excretion. We report a case of a 13-year old female with progressive familial intrahepatic cholestasis (PFIC) type 1 who demonstrated decreased uptake and excretion of gadoxetate disodium contrast material. This case illustrates the challenge of imaging children with cholestasis using hepatobiliary-specific contrast agents; we propose an alternative explanation for the delayed excretion that may be related to the underlying genetic defect of this child.