Kaplan-Meier analysis indicated that age of onset < 3mo (p = 0.0015, hazard ratio = 12.29, 95% confidence interval [CI] = 3.74-40.3), like liver failure (p = 0.0343, hazard ratio = 6.57, 95% CI = 1.96-22.0), conferred poor prognosis.<h4>Conclusions</h4>Early age of presentation, like liver failure, confers poor prognosis in ILFS1. Here, LARS1 is linked to liver failure.