Pulmonary arterial hypertension is a rare but progressive disease that leads to death. Modern drug treatment slows the progression of the disease and prolongs patients' lives, but often, even maximal treatment with parenteral prostacyclin does not prevent deterioration. In the case of inadequate clinical response to drug treatment, lung transplantation (LTx) should be considered. This article aims to analyze thoroughly indications to refer a patient for consultation with a transplant center, the optimal timing of listing for LTx, contraindications for the procedure, bridging techniques, as well as tests needed before and after transplantation. We outline the technique of the procedure and evaluate psychological aspects of LTx.