Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent, life-threatening bacterial and fungal infections of the skin, airways, lymph nodes, liver, brain, and bones. These infections most commonly occur in organs in contact with the outside world (lungs, gastrointestinal tract, and skin), as well as in lymph nodes that drain these structures. While involvement can be seen in many organs, there is no known cardiovascular involvement. Our case is an ACS case that has a different place in the literature because acute coronary syndrome (ACS) was seen in a 20-year-old male patient with a diagnosis of chronic granulomatous disease.