*Purpose: Liver transplantation (LT) was initially developed as a therapy for liver diseases known to be associated with a high risk of near-term mortality. As overall outcomes for the procedure have improved, LT has evolved into an attractive approach for a growing number of metabolic defects in a variety of clinical situations. Few studies have evaluated the long term outcomes of liver transplantation in this subset.

*Methods: Review of all pediatric transplants done for metabolic liver disease at our center over the recent 20 year cohort.

*Results: Thirty one patients received LT for metabolic defects. Median age was 4.4 years, 24 patients received whole livers and 7 patients partial livers. Indications are shown in table 1. The patient and graft survival were compared to 131 transplants performed during the same era for other liver diseases. (Figure 1). The patient survival for the patients with metabolic defects was 100% at 10 years.

*Conclusions: Our findings confirm that metabolic liver disease is a highly successful indication for liver transplantation with exceptional outcomes. The long term survival for children we report demonstrates a realistic expectation of close to normal life potential.

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