The clinical profile of 7 children and their follow-up is described. There was female preponderance with mate to female ratio of 1:6. The median age of onset was 6 years. All the patients had skin rash, muscle weakness and abnormal enzyme profile. Muscle biopsy was performed in 6 and was abnormal in all of them. The electromyogram (EMG) was performed in 6 and was found abnormal in five. All the children responded well to corticosteroids. Two children received intravenous dexamethasone bolus and showed good response.