Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a type of hypertrophic cardiomyopathy associated with left ventricular outflow tract stenosis. The increased pressure gradients across the left ventricular outflow tract in patients with HOCM could lead to circulatory collapse. We describe our experience with perioperative management under femoral nerve block (FNB), lateral femoral cutaneous nerve block (LFCNB), and transthoracic echocardiography (TTE) monitoring during open reduction and internal fixation of a femoral neck fracture in a patient with severe HOCM. Case presentation: A 72-year-old man, who was indicated to undergo open reduction and internal fixation of an intracapsular femoral neck fracture, had a history of treatment for hypertension and HOCM. He had heart failure for 4 years and was hospitalized several times. He was resuscitated after ventricular fibrillation and received an implantable cardioverter-defibrillator at that time. He also had severe physical limitations (New York Heart Association class III). We selected FNB and LFCNB as the methods for anesthesia and injected 0.25% levobupivacaine (20 mL) around the femoral nerve and 0.25% levobupivacaine (10 mL) into the lateral femoral nerve region. He underwent TTE during the perioperative period, which enabled us to perform hemodynamic and morphological evaluations of the heart. The intraoperative TTE findings remained stable from before the induction of anesthesia to the patient's exit from the operating room. Postoperatively, his hemodynamic parameters continued to remain stable. Conclusions: In this case, FNB and LFCNB contributed to hemodynamic stability during non-cardiac surgery. Additionally, TTE was useful for the perioperative evaluation of cardiac hemodynamics and morphology in our patient with severe HOCM.