Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that causes progressive degeneration of neurons in motor and non-motor regions, affecting multiple cognitive domains. In order to contribute to the growing research field that employs structural and functional neuroimaging to investigate the effect of ALS on different working memory components, we conducted a functional magnetic resonance imaging (fMRI) study exploring the localization and intensity of alterations in neural activity. Being the first study to specifically address verbal working memory via fMRI in the context of ALS, we employed the verbal n-back task with 0-back and 2-back conditions. Despite ALS patients showing unimpaired accuracies and reaction times, there was significantly increased brain activity of frontotemporal and parietal regions in the 2-back minus 0-back contrast in patients compared to controls. This increased brain activity was largely associated with a better neuropsychological performance within the ALS group, suggesting a compensatory effect. This study therefore adds to the current knowledge on neural correlates of working memory in ALS and contributes to a more nuanced understanding of hyperactivity during cognitive processes in fMRI studies of ALS.