Headaches in CADASIL are predominantly characterized by migraine with aura, often presenting before other neurological symptoms. These migraines are typically severe, featuring visual disturbances, sensory changes, and sometimes motor symp­toms, and they may be resistant to conventional therapies [1]. As CADASIL pro­gresses, migraines can evolve into chronic daily headaches, with tension-type headaches also emerging in later stages due to cerebral microangiopathy [5]. Migraine disorders occur in 20–40% of CADASIL patients, with some cases involv­ing status migrainous and persistent aura, highlighting the complex relationship between CADASIL and migraine [6]. The high prevalence of right-to-left shunt in CADASIL patients with migraines underscores the hereditary nature of the condi­tion and its pathophysiological links to headaches [5, 6]. Genetic mutations, particu­larly in the NOTCH3 gene, are closely tied to the manifestation of chronic headaches in CADASIL, emphasizing the importance of genetic screening [7]. Understanding these genetic factors is crucial for advancing diagnostic and therapeutic strategies, as CADASIL’s headache phenotype shares similarities with but diverges from typi­cal migraine with aura [7, 8]. Additionally, acute confusional migraine (ACM) may serve as an early indicator of CADASIL, often preceding diagnosis by several years [1, 2]. Current research underscores the need to reevaluate how CADASIL-related headaches are classied, advocating for a more nuanced approach considering the genetic mechanisms underlying these headaches [1].