Objective: To screen cognitive functions using the Montreal Cognitive Assessment (MoCA) test and to determine the most common central nervous system complications in adults with sickle cell anemia (SCA). Materials and Methods: One hundred adult patients with SCA and 82 healthy controls participated in this study. Controls were matched for age, sex, and education level. We reviewed the demographic information and laboratory values of all patients. The patients were questioned about common CNS complications including headache, ischemic or hemorrhagic stroke, epilepsy, and cerebral venous sinus thrombosis. The MoCA test was used to assess neurocognitive function in all participants. Results: Of the 100 patients with SCA, 38 patients had chronic or recurrent headaches, 10 had a history of depression, and four patients had a history of ischemic stroke. None of the patients had a history of epilepsy, hemorrhagic stroke or cerebral venous sinus thrombosis. The median MoCA score of the patients was significantly decreased compared with that of the control group (p<0.001). MoCA scores below 21 points were observed in 50% of the patients. The MoCA scores were negatively correlated with age but positively correlated with education level (r=-0.181 p=0.015, r=0.483, p<0.001 respectively). There was a significant correlation between a history of chronic or recurrent headaches and lower MoCA (p=0.003). Conclusion: Cognitive impairment was the most prevalent neurologic symptom in Turkish adult patients with SCA. The MoCA test may be a useful and easy screening test to evaluate and follow cognitive impairment. A history of first ischemic stroke during adulthood was observed in one patient. Two patients had severe neurologic sequela findings due to ischemic stroke.