Temporal arteritis (TA) is an inflammatory vascular disease common in the European population. It is mainly characterized by sudden onset headache. TA is rarely associated with other autoimmune diseases, such as Sjögren’s syndrome (SS). We present the case of a Peruvian 71 year-old man with SS history, who was admitted to the emergency department due to severe headache evolved in 4 days, periocular pain and right ptosis. The authors also performed a systematic review of case reports or case series of patients diagnosed with both TA and SS. This temporal arteritis case is an atypical presentation because headache was characterized by mixed nociceptive and neuropathic pain components. Despite the infrequency, new studies should be carried out to identify comorbidities in TA patients.