Key Clinical MessagePulmonary agenesis is a rare congenital abnormality. Patients with hemithorax white‐out on x‐rays should be treated with caution, especially in resource‐limited countries where chest CT and bronchofibroscopy are not available to confirm the diagnosis.AbstractPulmonary agenesis is an uncommon congenital abnormality defined by the complete absence of the lung parenchyma, as well as the bronchial and vascular structures. Right‐sided pulmonary agenesis is less frequent, has a worse prognosis, and is usually associated with other congenital abnormalities. We reported the clinical case of a 31‐year‐old woman with right pulmonary agenesis, and no other congenital abnormalities, whose diagnosis was confirmed by thoracic computed tomography and bronchofibroscopy and who has a good prognosis.