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Despite the introduction of NTBC into the treatment of tyrosinaemia type I (TTI) hepatocellular carcinoma (HCC) does occur in affected patients. Serial total a-Fetoprotein (AFP) levels are used to evaluate the individual risk to develop malignant changes. Lectin-reactive a-Fetoprotein is a recently described marker for early recognition of HCC in adult liver disease. Aim : To investigate if the analysis for Lectin-reactive a-Fetoprotein could lead to earlier detection of HCC compared to a judgement based on the evolution of total AFP alone. Patients : We report the analysis of AFP data from 41 patients with TTI. 12 patients had TTI and histologically proven HCC. Methods : AFP electrophoresis in a lectin containing agarose gel leads to separation of lectin-reactive and non-lectin- reactive AFP fractions. A proportion of more than 15% of lectin-reactive AFP is suspicious for the development of HCC in adult cirrhotic patients. Results : AFP subfractions could be identified in all 41 patients. In the patients with TTI and HCC in 6 patients the lectin reactive AFP was elevated before total AFP became abnormal, in 3 patients the rise in lectin reactive-AFP was consistant with the rise of the total AFP and in 3 patients the lectin reactive-AFP was raised after the total AFP or did not increase at all. Discussion : We were able to identify 6 out of 12 patients with HCC who had an early rise of lectin reactive AFP before they developed a change in total AFP levels. Conclusions : Analysis for lectin reactive AFP may allow early diagnosis of HCC in patients with TTI and thus facili- tate liver transplantation before the onset of metastasis. We suggest the further evaluation of lectin-reactive AFP in TTI.