Patients with PFIC3 were reported to be misdiagnosed as Wilson disease because of copper retention in some cases with cholestasis, that can result in flooding of copper in urine with penicillamine challenge test (Stalke et al. 2018; Gomez-Ospina et al. 2016; Qiu et al. 2017; Boga et al. 2015; Shneider 2011). The gene discussed is ABCB4; the disease is cholestasis.