BackgroundPulmonary sequestration (PS) is a congenital pulmonary malformation. Adenocarcinoma arising in PS is extremely rare.Methods and ResultsWe present the first reported case of synchronous intralobar PS and lung adenocarcinoma in the right lower lobe, which was successfully treated using robotic‐assisted thoracic surgery (RATS). The robotic system allowed for easy identification, clipping, and dissection of the abnormal artery, highlighting its benefits over traditional surgical approaches.ConclusionsThis case underscores the importance of considering the possibility of coexisting lung cancer in patients with a clinical diagnosis of PS and demonstrates the safety and efficacy of RATS in managing this rare condition.