One patient with ventricular fibrillation, dilated LV, and features compatible with left ventricular non-compaction cardiomyopathy (LVNC) had the common pathogenic RYR2 exon 3 deletion, c.(168 + 1_169-1)_(273 + 1_274-1)del, as a de novo variant. Here, RYR2 is linked to left ventricular noncompaction.