Mutations in transport and Golgi organization 2 homolog (TANGO2), which is located on 11.21 of chromosome 22 (22q11.21) and encodes a protein with the same name, were recently described as a cause of an autosomal recessive syndrome characterized by an episode of metabolic crisis associated with rhabdomyolysis, renal complications, cardiac arrhythmias, and neurodegeneration (OMIM 616878).1, 2. The gene discussed is TANGO2; the disease is cardiac arrhythmia.