Hypertrophic Cardiomyopathy (HCM) is a genetic heart disease characterized by left ventricular (LV) hypertrophy, with outcomes ranging from asymptomatic to sudden cardiac death (SCD). Accurate diagnosis and risk stratification are essential. This case report discusses a 52-year-old female with family history of sudden deaths, presenting with acute coronary syndrome symptoms. Investigations revealed atrial fibrillation, ST-elevation, and a thrombus in the left anterior descending artery (LAD). Echocardiography and cardiac CT confirmed a left ventricular apical aneurysm, multiple apical thrombus, and hypertrophic septal wall with systolic LV dysfunction. The report highlights the risk of thromboembolic events and SCD in patients with LV apical aneurysms. Meta-analyses and guidelines emphasize the importance of integrating risk markers like LV apical aneurysms and late gadolinium enhancement into clinical assessments. Given the patient’s family history, aneurysm, and LV dysfunction, primary SCD prevention with an implantable cardioverter-defibrillator (ICD) was deemed suitable. HCM patients with LV apical aneurysms represent a high-risk phenotype requiring comprehensive management, including SCD and stroke prevention.