Regarding PLEKHM2-deficient cardiomyopathy, Muhammad et al. [2] and Atkins et al. [3] respectively reported two cases of PLEKHM2 loss-of-function mutations leading to recessive DCM, one with a homozygous frameshift mutation and one with a compound heterozygous mutation consisting of a frameshift and a splice mutation. This evidence concerns the gene PLEKHM2 and cardiomyopathy.