A 70-year-old female patient was admitted to the hospital with complaints of dyspnea on exertion, fatigue, weight loss and palpitation lasting for one year but accentuated in the previous month. The radiological examinations revealed right middle lobe agenesis along with Macleod syndrome localized at the right inferior lobe. Macleod syndrome is a rare disease but the localization at the right inferior lobe accompanying a right middle lobe agenesis is even rarer. We present this case with an overview of the literature.