A two-year-old male child with global developmental delay was diagnosed with 3 methylglutaconic aciduria type 5 also called dilated cardiomyopathy and ataxia (DCMA) based on homozygous likely pathogenic variants in exon 5 of the DNAJC19 gene c.250C>T (pArg84Ter). Here, DNAJC19 is linked to Dilated cardiomyopathy with ataxia.