Background: The coronavirus disease 2019 (COVID-19) has been shown to affect several systems, notably the respiratory system. However, there has been considerable evidence implicating the nervous system in COVID-19 infection. This study aims to investigate the clinical characteristics of patients whose cerebrospinal fluid (CSF) tested positive for SARS-CoV-2.Methods: A comprehensive search of PubMed, EMBASE, Scopus, WHO Coronavirus database, bioRxiv, medRxiv, and Web of Science databases was carried out in August 2020. Original studies involving patients who tested positive for SARS-COV-2 in their CSF were included. Key search terms encompassed all variations of “COVID-19” AND “Cerebrospinal Fluid”.Results: A total of 525 studies were identified. 56 full-text articles were assessed, of which 14 were included. In total, 14 patients tested positive for SARS-CoV-2 in their CSF. 21.4% (3/14) of patients had negative nasopharyngeal (NP) swabs despite a positive CSF sample. 14.2% (2/14) of patients who initially had positive NP swabs developed neurological deterioration after a supposed recovery as indicated by their negative NP swabs, but their CSF still tested positive for SARS-CoV-2. Common symptoms were headache (42.8%; 6/14), fever (35.6%; 5/14), vomiting (28.6%; 4/14), cough (28.6; 4/14), visual disturbances (28.6%; 4/14), diarrhea (21.4%; 3/14), and seizures (21.4%; 3/14). Four patients (28.6%) were admitted to ICU, one (7.14%) was admitted to a rehabilitation facility, and two (14.3%) died.Conclusions: Physicians should be familiar with the presenting neurological features of COVID-19, and be aware that they can occur despite a negative NP swab. The results of this study are intended to aid in the development of informed guidelines to diagnose and treat COVID-19 patients with neurological manifestations.