This study aims to evaluate the prevalence, distribution and clinical profile of neurological diseases and syndromes from a neurology outpatient clinic in the city of Rio de Janeiro. This is a quantitative, cross-sectional study. 180 medical records of firsttime visits conducted through 18 months were analyzed. Age, gender and comorbidities were collected to identify the sample’s profile and the neurological diagnoses found were classified in predefined groups of syndromes according to the ICD-10 classification, which were further stratified according to age and gender. 157 (87,2%) of the patients had a neurological disease, of which the mean age was 51,05 years and 67,5% were women. The most frequent comorbidities were: hypertension (55,6%); dyslipidemia (20,4%); and diabetes (14,6%). The most common neurological diseases were: Sequelae of Cerebrovascular Disease (12,7%); Unspecified Dementia (8,9%); and Migraine without Aura (7,6%). The main neurological syndromes were: Headache (24,8%); Dementia (15,9%); Cerebrovascular Disease (13,4%); Movement Disorders (12,1%); and Radiculopathy (10,8%). The age and gender profile of these syndromes was further described. These data contribute to better understand the distribution of neurological diseases in the neurological outpatient setting.