Various neurological complications occur in primary or secondary antiphospholipid syndrome (APS) consisting of cerebrovascular attacks, ocular events, dementia, seizure, chorea, and transverse myelopathy that are all related to the titer of antiphospholipid antibodies (aPL). We report a patient with chorea and retinal vessel occlusion as manifestations of systemic lupus erythematosus (SLE) and APS. A 27-year-old man presented with progressive visual field defect and decreases visual acuity of right eye (OD) as well as involuntary movements in both hands and slurred speech. Investigations led to the diagnosis of SLE and APS. Anticoagulant and immunosuppressant was started for him. As his condition improved, the prednisolone was gradually tapered. This is the first case report of concomitant retinal vessel occlusion and chorea in SLE and APS.