Controversies in the occurrence and implications of headache in patients suffering from systemic lupus erythematosus (SLE) triggered us to conduct an extensive literature search in order to answer five clinical questions. (i) Is headache prevalence higher in SLE patients than in the general population? (ii) Is 'lupus headache' a separate entity? (iii) Is there a distinct pathogenetic mechanism of headache in SLE? (iv) Is headache related to CNS involvement or general SLE activity? (v) Is headache related to anxiety- and depression-like symptoms in SLE? All published articles reporting data from >30 SLE patients were classified into four classes (I, IIa, IIb and III) by the quality of their evidence. We found no prospective controlled study (class I), but we identified seven controlled (class II) and 28 uncontrolled studies (class III) that retrospectively investigated the occurrence of headache in SLE patients. Eight out of 35 studies applied the International Headache Society (IHS) criteria for headache classification, whereas only four uncontrolled studies investigated paediatric SLE populations (class III). Pooled data from eight studies (controlled and uncontrolled) that used the IHS criteria show that 57.1% of SLE patients reported any type of headache (migraine 31.7% and tension-type headache 23.5%). Pooled data from seven controlled studies showed that the prevalence of all headache types, including migraine, was not different from controls. Insufficient evidence was found for the concept of 'lupus headache'. No particular pathogenetic mechanism of headache in adult SLE patients has been identified, nor an association between headache and the disease status, including CNS involvement. There is no good evidence that headache is associated with anxiety and depression in SLE. Insufficient data (class III) do not allow safe conclusions for headache among paediatric SLE patients. These findings suggest that the occurrence of headache in adult SLE patients does not itself require further investigation and that headache in those patients should be classified according to IHS criteria and managed as primary headache if there is no specific indication of a role for SLE in the patient. These recommendations should be verified by a properly controlled and prospective study in both adult and paediatric populations.