Total leukonychia is an unusual finding in dermatomyositis. We present a juvenile dermatomyositis case with total leukonychia. Main observations: Our case, an 8 year-old female, has all the characteristic findings of amyopathic dermatomyositis including Gottron' s papules and heliotrope rash. The patient had symptoms constantly and she developed total leukonychia a year following the onset of dermatomyositis. Conclusions: To the best of our knowledge, this is the first case of dermatomyositis associated with total leukonychia.