Recently, a novel mutation in the Pleckstrin Homology And RUN Domain Containing M2 (PLEKHM2) gene, PLEKHM2[delAG], was linked to a rare and fatal cardiovascular disease called dilated cardiomyopathy with left ventricular non-compaction (DCM-LVNC). The gene discussed is PLEKHM2; the disease is left ventricular noncompaction.