Introduction: In patients with non-cirrhotic inborn errors of metabolism (IEM) metabolic stability drives the indication for liver replacement.  
Methods: A retrospective analysis of liver transplant (LT) recipients with non-cirrhotic IEMs from 2013-2023.
Results: Out of 187 LTs, 32 pLTS were destined to 27 non-cirrhotic IEM patients (9 female/16 male, with a mean age and weight of 64±50.3 months and 17.7±10.9 kg): 17 organic acidemias (13 propionic acidemias; 3 methylmalonic acidemias), 5 urea cycle disorders (1 citrullinemia; 1 argininosuccinic aciduria; 1 OTC deficiency; 3 arginase deficiencies), 4 Maple Syrup Urine Disease (MSUD), 2 ethylmalonic encephalopathies and one type 2 glycogen storage disease. 
Grafts were sourced from 5 (16%) living and 27 cadaveric donors: 10 whole-organ grafts (31%), 8 reduced cadaveric grafts (25%) and 9 split grafts (28%). Twelve aortohepatic conduits were employed to reconstruct hepatic inflow in 11 patients. Five recipients presented intraoperative loss of arterial flow secondary to vasospasm. 
During a 57.5±37.8 month follow-up, metabolic decompensation tapered and protein intake increased. A higher rate of arterial complications was noted in comparison to other LT indications (p<0.05). Three patients required retransplantation, the youngest was subjected to two additional re-do transplants. Two patients demised resulting in a patient survival rate of 97% at 1 year and 92% at 5 years.
Conclusion: Liver transplant improved metabolic stability and enabled diet liberalization in all IEM patients but at a higher rate of arterial complications when compared to other LT indications.