Among the 15 patients with skin rash, 12 (80%) presented with erythematous rash.<h4>Conclusion</h4>Erythematous skin rash may offer a clue to the diagnosis of anti-HMGCR myopathy in children with muscle weakness and serum creatine kinase level >5,000 IU/L in the absence of other myositis-specific antibodies, particularly in patients ≥7 years old. Here, HMGCR is linked to myopathy.