Background: Subarachnoid hemorrhage (SAH) is a rare complication of systemic lupus erythematosus (SLE). Methods: We made a retrospective search for patients with SLE and nontraumatic SAH from 1990 to 2006. Results: We found 10 patients with SLE and primary SAH of a total of 1,077 patients with SLE (0.93%); mean age of onset was 37.4 ± 15.25 years and the mean duration of SLE at the onset of SAH was 98.3 ± 50.32 months. SLEDAI and chronic damage scores were 3.67 ± 5.20 (n = 9) and 2.90 ± 1.45 (n = 10), respectively; 60% of patients had high Hunt-Hess scores and in only 50% of cases a saccular aneurysm was identified. Conclusions: SAH presents in about 1% of SLE patients. Long duration of SLE and chronic damage scores might be associated risk factors.