With each decade, the incidence of ischemic stroke among young people is increasing. After conducting a literature review, it was found that generally accepted causes of ischemic stroke relate more to people over the age of 45, while for young patients, the presence of hidden genetic pathology is more characteristic. The most common genetic causes are patent foramen ovale, arterial dissection, antiphospholipid syndrome, and hyperhomocysteinemia. In the clinical case under consideration, the patient experienced an ischemic stroke twice at a young age. The first stroke occurred due to uncontrolled hyperhomocysteinemia, the provoking factor of which was a sharp rise in temperature to 39.9 oC and a large amount of stress load on the body. The cause of the second venous cerebral infarction was low adherence to the prescribed medications, which led to the self-discontinuation of antiplatelet agents. We analyzed this clinical case and reviewed relevant literature on the mechanisms linking hyperhomocysteinemia to stroke. Key aspects of the management tactics for patients who have suffered an ischemic stroke against the background of hyperhomocysteinemia have been identified, which depends on the type of metabolic disorder: pyridoxine therapy, a lifelong low-protein diet, and the prescription of betaine. Primary care physicians are recommended to manage young patients with special attention to the specifics of the clinical presentation and adherence to therapy. The prospects for further research include the development of scales for identifying risk groups for hereditary pathology capable of leading to ischemic stroke at a young age, as well as the creation of unified, convenient, and effective methods for monitoring adherence to secondary prevention of acute neurological catastrophes and a system of active observation for this cohort of patients.