Few studies have examined the use of a short cognitive screening of ALS patients in order to establish which patients should undergo a more comprehensive neuropsychological assessment. We tested 20 patients with early ALS with four cognitive screening instruments and subsequently with an extensive neuropsychological assessment. Sixty percent of patients showed a deficit in at least two scores of tests administered, while 40% had three abnormal tests. Dysexecutive syndrome was the most common neuropsychological impairment. The Frontal Assessment Battery (FAB) proved a useful indicator of the presence of cognitive dysfunction to complete neuropsychological evaluation. In conclusion, the FAB can be considered a sensitive cognitive screening tool in these patients. These data will be verified on a larger sample of patients.