Background: Apical hypertrophic cardiomyopathy (AHCM) is a variant of hypertrophic cardiomyopathy affecting 25% of the Japanese population. Echocardiography is an essential method for the diagnosis and follow-up of this disease. Aim: The main objective of this study is to describe the prevalence and characteristics of AHCM in the Mexican population, who attended the National Institute of Cardiology Ignacio Chávez Material and methods: From 1994 to 2017, a total of 13 patients with AHCM diagnosis were included. All patients had complete medical history, electrocardiogram (ECG) and echocardiogram. In some patients, myocardial perfusion, nuclear magnetic resonance imaging (NMRI), pharmacological stress echocardiography, coronary angiotomography and cardiac catheterization were analyzed. Patients with other types of hypertrophic cardiomyopathy. Results: The mean age was 67.5±9.5 years, 62% were men. The clinical diagnosis for AHCM was on first instance in only 23% of the cases, the remaining 77% had an initial diagnosis of ischemic heart disease. Negative T wave was detected in 100% of the patients and 46.1% presented giant T-wave inversion. All of the patients were diagnosed by echocardiography, four patients presented a mixed form of AHMC and the rest of them pure AHMC. Conclusions: The prevalence of AHCM in our institution is of 0.4% in general population and in relation to hypertrophic cardiomyopathy (HM) of 3%. The clinical and electrocardiographic characteristics of AHCM suggested data compatible with ischemic heart disease. In all patients echocardiography should be the first-choice non-invasive study. In cases of diagnostic doubt or presence of concomitant diseases, other non-invasive imaging studies, such as CMRI or nuclear medicine are recommended.