Dropped head syndrome (DHS) is characterized by a passively correctable chin-on-neck deformity in erect posture and can stem from a wide variety of neurological disorders spanning the neuraxis. . Neuromuscular disorders account for a major chunk of DHS and include disease of anterior horn cell diseases, polyradiculopathies and cervical plexopathies, disease of neuromuscular junction and myopathies. Isolated DHS without additional neurological features poses a management challenge, particularly because the symptoms can significantly impact the patient's quality of life and may not always respond to treatment..(Ref)Here we present a patient with isolated DHS with evaluation revealing isolated next extensor myopathy with remarkable response to treatment. Although isolated neck extensor myopathy typically exhibit poor immunomodulatory response, timely identification and early intervention probably can lead to a favourable outcome in a subgroup of patients.