Dermatomyositis (DM) is a rare autoimmune disease characterized by non-infectious inflammatory damage to the skin and the predominant muscles in the belts. It is believed to be associated with about one case per 1,000 nasopharyngeal carcinoma (NPC). This association has been described for the locally advanced stage, stage II and stage III of the NPC. Little described in the early stages (stage I), its symptomatology is often indicative of cancer whose diagnosis is based on five criteria: muscle weakness of the proximal limbs, elevation of serum muscle enzymes, histological muscle abnormalities in muscle biopsy, electromyographic abnormality and clinical inflammatory dermatological manifestation. Treatment is based on corticosteroid therapy and etiological treatment of cancer. The prognosis of NPC in patients with paraneoplastic DM is comparable to non-infected patients. We report this case of DM revealing an early stage NPC and we take stock of the state of knowledge about paraneoplastic DM associated with rarely described NPC. Indeed, in Morocco the last description of a case dates back to 2016.