The dropped head syndrome (DHS) is a weakness of neck extension, leading to a typical presentation of the flexed head with the chin in contact with the chest wall. We report a case of a patient with DHS due to vasculitis of the CNS secondary to SLE and performed a brief review of the literature on the subject. SLE is a systemic disease with several neurological manifestations, the prompt recognition of these conditions usually leads to a better long-term prognosis from a functional point of view. We show the main complications and an example of a successful case due to the prompt care of the neurology and rheumatology.