GNE myopathy is a rare distal myopathy also known as hereditary inclusion body myopathy (HIBM) or Nonaka disease or Distal Myopathy with Rimmed Vacuoles (DMRV) or IBM2. It’s an autosomal recessive disease that can present in homozygous or compound heterozygous forms, with predominantly compound heterozygous mutations found in Indian subcontinent. Clinically most striking feature is distal leg weakness sparing quadriceps, distinguishing it with other common myopathies. GNE myopathy most commonly presents in the third decade of life as foot drop because of predominant Tibialis Anterior muscle involvement. Later it progresses to other muscles of lower and upper limbs; however Quadriceps usually remain spared even in advanced stages. Muscle biopsy typically shows rimmed vacuoles with atrophied fibres and congophilic depositions. Distal muscle weakness with sparing of quadriceps and rimmed vacuoles in muscle biopsy make this rare myopathy a unique one. Here we are presenting a typical case of GNE myopathy with founder mutation (p.Val727Met) which is common in Indian population.