CAPRIN1 is a protein crucial for mRNA translation and stability, which regulates brain development and synaptic plasticity and is essential for learning and memory. CAPRIN1 gene has been linked to rare neurological disorders. Indeed, a recurring missense variant is associated with early-onset ataxia, impairing coordination, and movement control; conversely, loss-of-function variants lead to a neurodevelopmental disorder, highlighting CAPRIN1’s pivotal role in brain function. Understanding CAPRIN1’s involvement in these conditions will shed light on its significance in maintaining normal brain function and the consequences of its dysfunction. Further research into CAPRIN1’s mechanisms in both physiological and pathological states is crucial for developing potential therapeutic interventions for these neurological disorders.