Introduction: Pulmonary agenesis is a rare congenital anomaly. Other cardiovascular, gastrointestinal, musculoskeletal, and urogenital system anomalies can be observed in association with it.
Case Report: A 24-year-old female patient presented to our clinic with a history of cough and chest pain with an abnormal chest X-ray. Physical examination was normal. Chest X-ray, CT-Scan and endoscopy led to the diagnosis of right upper lobe pulmonary agenesis.
Conclusion: The diagnosis of pulmonary malformations like agenesis or hypoplasia can be delayed and may not occur until adulthood. Once the diagnosis has been established medical follow up is mandatory.
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