The authors relate a unique observation of the familial form of proximal 
myodystrophy with early contractures and malignant course. The primary character 
of muscular injury was confirmed on electromyography. The data of 
electrocardiography and echocardiography attested to the presence in the 
patients of the signs of cardiomyopathy. Since the disease was diagnosed in 3 
brothers, the X-coupled recessive type of its inheritance is assumed. An opinion 
is advanced that the described form is a clinical variety of Emery-Dreyfus 
myodystrophy.