Apical variant hypertrophic cardiomyopathy (AHCM) is characterized by asymmetric hypertrophy of the left ventricular (LV) apex. T wave inversions of variable degree, particularly in the left precordial leads, and left ventricular hypertrophy (LVH) are common EKG findings in AHCM. Echocardiography is typically the initial imaging modality used in the diagnosis and evaluation of AHCM. The diagnosis is made when the LV apex has apical wall thickness of ≥ 15 mm or a ratio of apical to basal LV wall thickness of ≥ 1.3 at end-diastole. The use of microbubble contrast agents with echocardiography is helpful for visualization of the apex. Cardiac magnetic resonance (CMR) has the advantage of a large field of view and the ability to perform tissue characterization. Late gadolinium enhancement (LGE) sequences are essential in the assessment of potential areas of myocardial scarring. Cardiac computed tomography (CCT) has the advantage of being able to evaluate coronary arteries in addition to assessing cardiac anatomy and function. A “Solar Polar” map pattern is the characteristic feature of AHCM on myocardial perfusion imaging (MPI) in cases not associated with apical aneurysm (APA). Recognition of typical perfusion patterns in AHCM patients is not only important in the diagnostic evaluation of this disease process, but also for avoiding unnecessary and costly tests. The purpose of this article is to review the imaging features of AHCM from different imaging modalities and assess the value added of each modality in the diagnosis of AHCM.