Here, we present a young child with early ischemic stroke, intractable epilepsy, and significant global developmental delay due to a novel de novo variant, p.Leu1692Gln in CACNA1A. As discussed, variants in this gene have been implicated in a wide range of neurologic phenotypes and well‐described disorders, such as FHM1, EA2, and SCA6 (Byers et al., 2016). The gene discussed is CACNA1A; the disease is epilepsy.