Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a cardiac condition of an abnormal interventricular septum (IVS) thickening. It is due to an autosomal dominant mutation in sarcomeric protein genes. It had many phenotypes with different distribution of myocardial hypertrophy. Septal myectomy remains the treatment of choice for hypertrophic obstructive cardiomyopathy resistant to medical therapy. However, controversy exist regarding standard treatment option(s). Description of the Case: Here we described a case of 47-year-old patient admitted with diagnosis of acute coronary syndrome, with typical chest pain of angina class II, shortness of breath class-II (NYHA), smoker on regular renal hemodialysis.  Echocardiography showed HOCM, IVS thickening, normal Left ventricle global systolic function and mild to moderate eccentric regurge. Coronary angiography revealed normal left main with total (LAD) proximal occlusion, proximal and mid (LCX) (50%) occlusion, first (OM) mid (90%) lesion, (RCA) dominant with non-flow limiting lesions. He was submitted to open heart surgery with four grafts, sub-valvular myomectomy, and successful myomectomy. Distal anastomosis was done first then myomectomy then proximal anastomosis. Patient extubated after (9) hours with stable hemodynamics and complete neurological condition, with accepted drains output. He gradually weaned from inotropic support and chest drains removed on 3rd postoperative day. All lines and pacemakers were removed, the wound was dry with stable sternum and improvement of all symptoms. Then discharged home after 9 days of hospital stay on medical treatment. Postoperative echocardiography showed significant improvement.   Conclusion: Surgical treatment is the standard option in treatment of HOCM to improve symptoms.