<h4>Purpose</h4>This study aims to characterize the clinical and genetic features of a sporadic case of dilated cardiomyopathy (DCM) associated with a TNNT2 variant and to review the variant spectrum of the TNNT2 gene in the Chinese DCM population.<h4>Patients and methods</h4>A 41-year-old male proband diagnosed with DCM underwent comprehensive clinical evaluation. Here, TNNT2 is linked to familial dilated cardiomyopathy.