Fanconi anemia, an autosomal recessive and X-linked disorder, is known to be associated with a variety of neoplasms. Liver tumors are one of the most frequently observed neoplasms but the association between the two disorders remains obscure. We present a case of a 27-year old female Fanconi anemia patient diagnosed with a mass on the right lobe of the liver measuring 90xx75xx60 mm. Histopathological examination of the mass after right hepatic lobectomy revealed focal nodular hyperplasia. This appears to be the first reported case of a hepatic focal nodular hyperplasia of such proportion associated with Fanconi anemia. Previously reported cases of liver tumors in association with Fanconi anemia in the English Literature were either hepatocellular carcinomas or hepatic adenomas.