Over the years, knowledge regarding the natural history of Chiari malformation type I (CM-I) has improved. However, there are still uncertainties in the literature regarding asymptomatic and oligosymptomatic patients with CM-I. We performed a literature review in order to determine the natural history of CM-I in symptomatic patients who were not operated and in asymptomatic adult patients. Among symptomatic patients, the most frequent symptom was headache (77%). Cough-related headache alone (34%), migraine-related headache alone (21.4%), and both cough- and migraine-related headaches (21.8%) were the most prevalent forms of headache. Some symptoms improved more effectively with surgical treatment options; cough-related headaches improved by 95% following surgery and by 40% following conservative treatment. Other headache types improved by 93% and 61.5% following conservative and surgical treatments, respectively. Patients diagnosed with asymptomatic or oligosymptomatic CM-I may improve over time or stabilize with conservative treatment. There is great heterogeneity in the literature regarding the diagnostic criteria for CM-I. Owing to the heterogeneity among the articles identified for this review, there is no consensus regarding the precise indications for surgery in asymptomatic patients. The natural CM-I history in asymptomatic patients reveals a favorable evolution.Copyright © 2020, Dantas et al.