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BackgroundDermatomyositis (DM) is a systemic autoimmune disease characterized by distinct skin lesions and a clinically heterogeneous constellation of systemic manifestations. This disease poses a challenge to clinicians because of its rarity, diverse clinical presentations, and variable organ involvement, resulting from an autoimmune attack on affected organs, which could be triggered by environmental factors in genetically susceptible individuals. Renal involvement is rare, with immunoglobulin M (IgM) nephropathy yet to be reported in patients with DM.Case presentationA 38‐year‐old man was admitted to Shariati Hospital, affiliated with Tehran University of Medical Sciences, with proximal weakness of the upper and lower extremities that had developed in the preceding month after receiving the Sinopharm COVID‐19 vaccine. The patient was diagnosed with DM based on the heliotrope rash, Gottron's papules, progressive proximal muscle weakness, and paraclinical findings. IgM nephropathy developed subsequently, diagnosed by light and immunofluorescence microscopy.ConclusionWe describe the first case of IgM nephropathy in a DM patient following COVID‐19 vaccination. This phenomenon requires further investigation into the possible crosslinks between the pathogenesis of IgM nephropathy with DM and the COVID‐19 vaccine. Diagnosing renal complications in DM patients promptly and accurately can help to achieve the best outcomes.

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