The hypertrophic cardiomyopathy phenotypes, including left ventricular outflow tract obstruction as well as diastolic and systolic heart failure, are encountered periodically in the critical care setting. An understanding of diagnostic evaluation and therapeutic management in the acutely ill patient with HCM will enable caregivers to optimize cardiovascular outcomes. This chapter will discuss management of several acute care scenarios by which HCM patients may present, including medical therapy as well as more advanced options. In addition, the rapidly expanding field of percutaneous intervention for structural heart disease offers novel therapy for outflow obstruction in addition to traditional surgical intervention. Acute management of potential complications following structural heart disease procedures will be discussed. Finally, case studies will be presented to provide a real-world experience of high-risk HCM patients in the acute setting.