ABSTRACT Pulmonary arterial hypertension (PAH) is a life‐threatening condition characterized by significantly elevated pulmonary artery pressure (PAP) and resistance. While targeted drugs can alleviate symptoms, their long‐term efficacy is limited, and treatment is often associated with notable side effects and financial burden. Percutaneous pulmonary artery denervation (PADN) offers a novel interventional approach for PAH patients with inadequate responses to targeted therapy by selectively ablating sympathetic nerves and restoring neurohumoural balance. This case report describes a 67‐year‐old male with severe PAH who underwent PADN after showing a poor response to long‐term macitentan and tadalafil therapy. Immediate postprocedure haemodynamics revealed a reduction in mean PAP (mPAP) from 42 mmHg to 32 mmHg and a 16% decline in pulmonary vascular resistance (PVR; 10.10 to 8.48 Wood units). A 6‐month follow‐up demonstrated sustained improvements in the 6‐min walk distance (6MWT), WHO functional class, Borg dyspnoea index, and NT‐proBNP levels. Clinical studies have confirmed that PADN can significantly improve the haemodynamics and exercise tolerance of medium‐ to high‐risk PAH patients by inhibiting excessive sympathetic nervous system activity and promoting pulmonary vasodilation, especially when combined with targeted drugs, which can produce synergistic effects. However, large‐index trials are needed to validate its long‐term efficacy, expanded indications, and procedural standardization.