Apical hypertrophic cardiomyopathy (ApHCM) is a less common type of hypertrophic cardiomyopathy (HCM) and is often characterized as benign. However, more recent publications indicate that there is significant heterogeneity among individuals with ApHCM and that for many the condition is less than benign. This review will discuss the most recent understanding of ApHCM in the context of a case presentation and will provide a basic framework for risk stratification.