These observations place EPG5-related Vici syndrome within an emerging group of early-onset neurodevelopmental and neurodegenerative disorders associated with defective autophagy (reviewed in Ebrahimi-Fakhari et al., 2014, 2015) such as static encephalopathy in childhood with neurodegeneration in adulthood (SENDA) due to recessive mutations in WDR45 (Haack et al., 2012), and a recently recognized syndromic form of early-onset ataxia due to recessive mutations in SNX14 (Thomas et al., 2014; Akizu et al., 2015). The gene discussed is SNX14; the disease is neurodegeneration with brain iron accumulation 5.