Therefore, a wide investigation is necessary to diagnose this pathology, including an anatomopathological study and genetic tests.<h4>Methods/results</h4>The purpose of this study is to report about a patient who had restrictive cardiomyopathy due to mutation on Filamin C gene and was indicated for heart transplantation.<h4>Conclusion</h4>The etiology of cardiomyopathy is important for the clinical management of the patient and also for guiding families regarding genetic counseling and prevention of new cases in the family. This evidence concerns the gene FLNC and restrictive cardiomyopathy.