Although it is easy to understand that mutations in RYR1 or CACNA1S can be found in patients with MH or CCD and that mutations in STIM1 or ORAI1 are associated with TAM/Stormorken syndrome, less obvious are the cases where mutations in genes not involved in Ca2+-handling pathways, such as MYH7, TTN, or MEGF10, are detected in patients presenting with clinical symptoms and histopathological alterations like those present in RYR1-related myopathies. The gene discussed is ORAI1; the disease is Stormorken syndrome.