We report a case of agenesis of the right lobe of the liver, which was demonstrated by imaging studies, in a patient with epiglottis carcinoma with the metastases to the cervical lymph nodes and liver. A 62-year-old male was admitted to our hospital because of a right cervical tumor. All laboratory findings, including those related to the hepatic function, were within normal limits. Abdominal ultrasonography and CT scanning showed the absense of the liver in the postero-lateral area to the Cantile line. The left lobe of the liver was markedly enlarged. Ultrasonography revealed only one hepatic vein. Abdominal angiography showed the absence of the right hepatic artery and right branch of the portal vein. The patient was diagnosed as agenesis of the right lobe of the liver by these imaging studies.