Swyer-James-Macleod syndrome (SJMS) is a rare etiology of a unilateral hyperlucent hemithorax but an important one, which should be considered in any individual with such findings. Presentation usually occurs in adulthood with an asymptomatic history in many cases or with a history of childhood infections. Clinically, symptomatic patients may present with productive cough, dyspnea on exertion, hemoptysis, decreased exercise tolerance and recurrent pulmonary infections. Many individuals are asymptomatic only requiring conservative management. However, some patients may require surgical intervention. SJMS can be easily misdiagnosed and must be suspected for example, in any patient diagnosed with asthma who does not respond to therapy. We report a case of a 51-year-old African American male diagnosed with SJMS on the basis of his medical history, clinical presentation and x-rays and computed tomography chest scans findings.