Barth Syndrome (BTHS; MIM 302,060) is a rare X-linked disorder caused by defects in the gene TAFAZZIN that is characterized by multisystem involvement including: prenatal onset of left ventricular non-compaction, cardiomyopathy, intermittent neutropenia, skeletal myopathy, growth impairments and other features [1–5]. The gene discussed is TAFAZZIN; the disease is Barth syndrome.