Dropped head syndrome (DHS) occurs in patients with severe weakness of the neck extensor muscles, of diverse origin, but most frequently because of neuromuscular diseases. Exceptionally, DHS may be the first manifestation of a systemic autoimmune disease. This report describes the case of a patient with DHS as a presenting sign of scleromyositis. We analyze the clinical features of the case and discuss the most important aspects of its pathogenesis and differential diagnosis.