To date, only two mutations have been identified in DNAJC19 (DnaJ/Hsp40 homolog, subfamily C, member 19) that are associated with dilated cardiomyopathy with ataxia (DCMA; Davey et al., 2006; Ojala et al., 2012), an autosomal-recessive disorder that presents with early onset dilated cardiomyopathy, non-progressive cerebellar ataxia leading to motor delays, testicular dysgenesis, growth failure, and elevated levels of 3-MGA. Here, DNAJC19 is linked to 3-methylglutaconic aciduria type 5.