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Background. Heliotrope rash is one of the characteristic skin manifestations of juvenile dermatomyositis. It is a reddish-purple rash on the upper eyelids that is usually bilateral. Case Presentation. We report a boy who presented with unilateral heliotrope rash, Gottron’s papules, and muscle weakness. Muscle biopsy was consistent with inflammatory myositis. Patient was started on prednisolone and methotrexate with an excellent response in both the skin and muscles. Conclusion. Unilateral heliotrope rash can occur in patients with juvenile dermatomyositis. Being a paraneoplastic condition caution should be taken not to miss any underlying malignancy.