The Emery-Dreifuss muscular dystrophy is a form of muscular dystrophy that 
frequently presents early contractures and cardiac conduction defects, caused by 
emerin deficiency in the inner nuclear membrane of the muscular fibers. A 
19-years-old man it presented muscle weakness and hypotrophy in the proximal 
upper and lower limbs, dysphagia and early contractures in elbows and ankles, 
with familiar history compatible with X-linked inheritance form. The 
investigation showed increased serum creatinekinase levels electrocardiogram had 
a first degree atrioventricular block and right bundle branch block normal 
electromyography and nerve conduction study muscle biopsy disclosed myopathic 
characteristics and nuclear protein immunohystochemical analysis showed 
deficiency of emerin. The clinical and genetics manifestations, laboratorial and 
electromyography changes, as well as, the study of the pattern of inheritance 
for genetic counseling are discussed.