Tyrosinemia could arise from both liver failure of any causes or from an inborn error of tyrosine metabolism and mainly lead to death in case of no treatment. The best choice of treatment especially in those with critical conditions is liver transplantation. In this study we report the outcome of liver transplantation in patients with tyrosinemia in our institution. Between January 2007 and March 2008, 9 patients with tyrosinemia and liver cirrhosis, which were confirmed by detecting plasma succinylacetone, have been receiving liver transplant­ation. Seven of 9 patients had received livers from living donors and the rest from deceased donors. Two patients were re-operated due to post-operative internal bleeding, and one patient developed wound infection. The mean duration of hospital stay in these patients was 17± 6.02 days. Diagnosis of acute rejection was made for 4 patients clinically and biochemically and have been treated with pulse of methyl prednisolone. One patient developed post transplant lympho­proliferative disorder and died. In conclusion, Despite of some complications, liver transplantation in our patients as the same as the other reports could be presented as an effective treatment for tyrosinemia resulting in clinical and biochemical improvement with good quality of life.