Hepatocellular carcinoma (HCC) is rare in children. Progressive familial intrahepatic cholestasis type II (PFIC2 and alsocalled BSEP ((Bile Salt Export Pump)) deficiency) is an inherited disease that initiates end-stage liver cirrhosis which canpredispose to HCC. HCC can occur in 15% of patients with PFIC2. In this case report, an 11-month-old boy with PFIC 2 wasadmitted for liver transplant work up. The finding of HCC was made incidentally by histopathology on the explanted liver aftersuspiciously gross examination. In this article, we found that the radiology (US) alone is not enough to exclude HCC. Finally,we conclude that any case of PFIC 2 (male or female) needs routine screening of serum AFP concentration, advancedradiological examination (CT, MRI) as well as careful macroscopic examination of their explanted liver (triple assessment) toexclude HCC.