Dysferlinopathy refers to myopathy due to defective dysferlin protein located in the sarcolemma. The major phenotypes include limb-girdle muscular dystrophy type 2B and Miyoshi myopathy. However,in recent years substantial symptomatic variations as well as some minor atypical phenotypes have increasingly been recognized. In this work, the authors detected concomitant atrial flutter and anti-centromere antibody in a biopsy-confirmed late onset dysferlinopathy patient. The authors also discuss cardiac manifestation of dysferlinopathy and the relationship between defective dysferlin and autoimmune processes in dysferlinopathy patients.