Strategies for therapy are based on whether a given CMS  modified the synaptic response to acetylcholine .At the same time we should rule out polymyositis, stiffperson syndrome ,neuromyotonia and  myopathic disorders.

We are presenting a case of young male bornout of nonconsanguineous marriage  who presented with progressive  limb –girdle weakness without family history.

Abbreviation –CMS–congenital myasthenic syndrome