We report four patients with "dropped head syndrome," a recently described nonprogressive myopathy characterized by severe neck extensor weakness. This relatively benign condition may be confused with more ominous neuromuscular disorders that also present with prominent neck weakness. We compared clinical and laboratory data from the patients with dropped head syndrome with findings from patients with head drop caused by other neuromuscular conditions. Patients with "isolated neck extensor myopathy," a term we prefer to "dropped head syndrome," could be readily identified with electrophysiologic, radiographic, and histologic studies.