<p>Cerebral amyloid angiopathy (CAA) is associated with transient focal neurological episodes (TFNEs), which are brief, stereotyped symptoms. As these may occur regularly, pharmacological treatment of TFNEs is needed but remains poorly investigated. To systematically evaluate the available evidence on the use of pharmacological treatment to reduce the frequency, severity, or duration of TFNEs in patients with CAA. A systematic literature search of PubMed and Embase was conducted. Eligible studies included observational studies, interventional trials, and case reports or series that assessed pharmacological interventions for TFNEs in patients with confirmed CAA. Nine case reports/series involving 13 patients met the inclusion criteria. The mean age was 75.15&#xa0;years, with a predominance of sensory and motor TFNE symptoms. One EEG recording out of nine reported paroxysmal activity whereas the rest showed no epileptiform activity. Antiseizure medications (levetiracetam) were the most common treatment. Eleven patients experienced complete remission, and two had partial improvement. Most patients exhibited convexity subarachnoid hemorrhage or cortical superficial siderosis on imaging. Although symptomatic improvement following pharmacological treatment was commonly reported, the current evidence is limited to descriptive case reports without control groups. The unpredictable course of TFNEs and absence of long-term follow-up complicate treatment evaluation. Larger, controlled studies or observational studies are needed to establish effective and evidence-based treatment strategies for TFNEs in CAA.</p>