Microembolic signals (MES) are detected by transcranial Doppler (TCD), and are mainly observed in cardiac and large-artery diseases; however they might also be observed in conditions affecting small vessels of the brain. We aimed to review the current medical literature related to MES assessed by TCD in patients with cerebral small vessel disease. We conducted a systematic review in PubMed and selected the articles with information on this topic. Systemic lupus erythematosus (SLE) is the disease with more articles; pooled data showed a frequency of MES of 14.9%. MES were more frequent in SLE patients with antiphospholipid antibodies and ischemic stroke. MES have also been described in other diseases such as primary antiphospholipid syndrome, Sneddon's syndrome, and Behçet's disease. Further studies assessing the significance of MES in the pathogenesis of neurological manifestations of these disorders is warranted.