A 62 years old male with a slowly progressive focal myopathy is presented. He had noticed weakness in the lower limbs for three years and weakness in the upper limbs for one year. He had bilateral atrophy of quadriceps and biceps muscles, absence of knee jerks and hypertrophy of the calves. Needle EMG showed myopathic motor units. Hystological study was compatible with muscular dystrophy. The clinical and laboratory characteristics of this patient are in keeping with what has been described as "Quadriceps Myopathy" as a form of a muscular dystrophy.