The prominent or isolated weakness of cervical extensor muscles is a relatively rare clinical sign. Commonly, this is known as “dropped-head syndrome”. This abnormal flexion of the head may occur in a variety of neuromuscular diseases and in a few non-neurological disorders as well. Systemic sclerosis is a clinically heterogeneous disorder which affects small arteries, microvessels and connective tissue with the involvement of multiple organs such as lung, heart, kidney and gastrointestinal tract. There is no evidence in literature of association between dropped head syndrome and rheumatic diseases, particularly systemic sclerosis.

The case we describe concerns a 74-year-old woman with dropped-head syndrome associated to Systemic sclerosis and pulmonary hypertension in absence of myositis signs.