A rare case of coronary artery bypass grafting in a patient with coronary artery disease and neurofibromatosis type 1 is presented in the article. The interest of this case is caused by small incidence of concomitant disease. The efficiency of coronary artery bypass surgery in a patient with this pathology is shown. Clinical pattern and echocardiography data confirmed improvement of patient’s condition and reduced functional class of angina pectoris despite occlusion of internal mammary artery. This case report emphasizes advisability of coronary bypass surgery in these patients and need for further research and accumulation of such clinical cases.