A 61-year-old Chinese man was diagnosed with liver cancer and diffuse intrahepatic and abdominal lymph node metastasis by abdominal magnetic resonance (MR) in May 2017, and subsequently confirmed as ICC (stage IV) with IDH mutation (IDH1-R132C) by biopsy and gene sequencing. Here, IDH2 is linked to intrahepatic cholangiocarcinoma.