BACKGROUND CVST (cerebral venous sinus thrombosis) may sometimes be associated with autoimmune disorders that require specific treatment. The clinical and magnetic resonance imaging (MRI) findings of systemic lupus erythematosus (SLE) patients with CVST are presented and contrasted with CVST without SLE. METHODS Consecutive patients with CVST admitted in neurology service during 2012-2016 were included. The diagnosis of CVST was confirmed by MR venography or digital subtraction angiography. SLE was diagnosed according to American College of Rheumatology criteria. The clinical and MRI findings of CVST with SLE and those without SLE were compared. RESULTS Forty-three consecutive patients with CVST were included during the study period, 3 of whom had SLE .Their age ranged between 20 and 35 years and all were females. The clinical markers of SLE were present in all and included oral ulceration in 3 patients, serositis in 2 patients, and arthritis and psychosis in 1 patient. The SLE patients did not have antiphospholipid antibodies or lupus anticoagulant. The manifestation of CVST in SLE was similar to the other patients with CVST. The CVST in SLE required long-term anticoagulation and immunosuppression with cyclophosphamide pulse in 1 patient and oral prednisolone in 2 patients. The outcome was good, partial, and poor in 1 patient each. CONCLUSIONS CVST may be the presenting feature of SLE, but these patients often have clinical clues to SLE. These patients need prolonged anticoagulation and immunosuppression.