ObjectiveTo analyze the clinical features of Becker muscular dystrophy（BMD）with dilated cardiomyopathy，and to raise medical workers’ awareness of the disease. 〖WTHZ〗Methods Collect the clinical data of a case of Becker muscular dystrophy with dilated cardiomyopathy admitted to the Department of Neurology of Zhengzhou University in October 2019 were and follow up. Analyze the clinical manifestations，imaging features，histopathology and genetic test results retrospectively. Results The patient’s clinical manifestations：muscular symptoms such as weakness of the limbs，muscle pain，intolerance to movement and manifestations of dilated cardiomyopathy of chest tightness，palpitation，wheezing，exacerbation after activity. Muscle biopsy and immunohistochemistry：Dystrophin-N（-），Dystrophin-R（membrane +），suggesting Becker muscular dystrophy. Genetic examination：DMD gene exon 10～18 deletion caused by Becker muscular dystrophy. Conclusion For male patients with dilated cardiomyopathy with limb weakness as the primary factor and significantly increased muscle enzymes，the possibility of Becker muscular dystrophy should be considered. For dilated cardiomyopathy caused by BMD，ACEI and β-blockers are effective.