Background: Pulmonary hypertension (PH) is a rare disease often associated with high mortality and is recently recognized as a common complication secondary to chronic kidney disease. Macitentan can improve clinical outcomes in patients with pulmonary artery hypertension in clinical trials. To observe the clinical efficacy and safety of macitentan tablets in the treatment of hemodialysis HD patients complicated with PH. Methods: This was a retrospective study performed in the renal division of Shaanxi Provincial People's Hospital between January 1, 2024, and December 31, 2025. A total of 10 patients with PAH who underwent regular hemodialysis (HD) (three times weekly for 4 hours each session) were enrolled. All patients received basic treatment, based on which macitentan tablets were added (10 mg per dose, once daily, oral administration), with a treatment cycle of 12 weeks. The 6-minute walk distance (6MWD), pulmonary artery systolic pressure (PASP) measured by echocardiography, and WHO functional class were compared before and after treatment. Serious adverse events (SAEs) and adverse drug reactions (ADRs) of macitentan were collected. Results: After 12 weeks of treatment, the proportion of patients with WHO-FC II increased from 20% to 60% (p = 0.046). LVEF showed an upward trend, with the median rising from 50.5% to 53.5% (p = 0.03). The mean 6MWD increased by 28.8 m (p = 0.035). Median NT-proBNP decreased from 39018 ng/L to 18987.5 ng/L (p = 0.017). Meanwhile, mean PASP dropped from 52.1 (5.363) mmHg to 39.4 (8.796) mmHg. Only two mild adverse reactions (dizziness and aggravated mild anemia) occurred during the entire observation period, which were relieved after symptomatic treatment. No severe liver or renal function impairment or treatment discontinuation was observed. Conclusion: With regular HD and basic treatment, macitentan demonstrates clear short-term efficacy and controllable safety in HD patients with WHO class II-III PH.