BACKGROUND:Dermatomyositis is an inflammatory myopathy that has an increased risk of malignancy, warranting aggressive health maintenance screenings. Dermatomyositis can rarely present with vulvovaginitis, thus clinical suspicion is important in early diagnosis.CASE:We present the case of a 31-year-old woman with a 10-year history of vulvovaginitis as her presenting symptom of dermatomyositis. On further investigation, she had a history of joint pain, muscle pain, fatigue, and leukopenia. On examination, she was found to have a rash and mucopurulent vulvovaginitis. Biopsies from the chest, axilla, and vulva were compatible with dermatomyositis. Evaluation for malignancy was negative, and her symptoms resolved with treatment of the dermatomyositis.CONCLUSION:Dermatomyositis is an inflammatory disease that can be associated with malignancy. This particular patient presented with vulvovaginitis. This case is important because the diagnosis was delayed. Early recognition is important to evaluate for malignancy.