Here, we present the case of a 4‐year‐old female with a history of recurrent ischemic strokes starting at 6 weeks of age, intractable epilepsy, and significant global developmental delay who was found to have a novel likely pathogenic, de novo variant in CACNA1A (p.Leu1692Gln). The gene discussed is CACNA1A; the disease is ischemic stroke.