Chronic hypoxia can lead to gain of function somatic variants in the <i>EPAS1</i> gene that encodes for hypoxia-inducible factor 2-alpha (HIF-2α), involved in PPGL tumorigenesis.<h4>Objective</h4>To describe a rare case of PCC in a pulmonary transplant patient and characterize the tumour's genetic background.<h4>Clinical case</h4>A 47 year-old man underwent a lung transplant for chronic obstructive pulmonary disease associated with alpha-1 antitrypsin deficiency. The gene discussed is EPAS1; the disease is chronic obstructive pulmonary disease.