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Congenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous connection presenting as episodic retrosternal chest pain on exertion in an adult has not been described. A 21-year-old female, non-smoker, presented with a 4-year history of sharp, episodic, retrosternal chest pains brought on with exercise. A contrast-enhanced computed tomography (CT) scan showed a grossly overinflated left lower lobe with partial anomalous pulmonary venous drainage into the left hemi-azygos vein. Lobectomy, the recommended treatm