A 14‐year‐old boy (patient 79.1), compound heterozygous for EPG5 variants p.Gly2231Val and c.4474+1G>A, presented in adolescence on the background of moderate neurodevelopmental delay with a complex movement disorder of subacute onset, including prominent parkinsonism, generalized dystonia with orofacial dyskinesia, and intermittent jerky action tremor. This evidence concerns the gene EPG5 and movement disorder.