Ataxia telangiectasia (A-T) is a rare autosomal recessive disease characterized by ataxia, cutaneous and ocular telangiectasia, impaired immunity with susceptibility to sino-pulmonary infections, radiation sensitivity and cancers particularly of haemato-lymphoid origin. Liver function tests abnormalities and elevated alfa feto-protein have been reported in A-T however there is no reported case of combined hepatocellular-cholangiocarcinoma (cHCC-CC) in literature. These tumours should be treated in similar fashion as in general population however reduction of chemotherapy dose might be helpful in decreasing chemo-toxicity.