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<bold>Background:</bold> Positive airway pressure (PAP) treats sleep-related breathing disorders (SRBD) in obesity hypoventilation syndrome (OHS). This study assessed long-term survival in stable OHS patients on PAP and the influence of adherence. <bold>Methods:</bold> Stable OHS patients newly diagnosed at University Clinic Golnik from 2005 to 2010 were included, with SRBD-free patients as controls. Medical data were gathered from hospital records, and vital status was censored at 13 years. <bold>Results:</bold> The sample included 115 OHS patients and 157 controls. OHS patients were older (56.9 vs. 49.8 years) and had higher rates of arterial hypertension (74% vs. 28%), chronic heart failure (26% vs. 3%), and diabetes (24% vs. 7%), all p<0.05. Comorbid OSA was present in 84% of OHS patients; 81% received CPAP initially, 8% BIPAP, with 64% adhering to PAP. Survival was lower in OHS patients (66%) than in controls (92%), p<0.001. Among OHS patients, survival was higher in adherent (75%) than non-adherent (50%) patients, p=0.003. In Cox regression adjusted for sex, age, pCO2, pO2, AH, CHF, diabetes, BMI, and non-adherence, age (HR 1.06, CI 1.02–1.11) and non-adherence (HR 2.5, CI 1.22–5.12) predicted mortality in OHS patients. In a similar model including only adherent OHS patients and controls, with OHS diagnosis included as a variable, age (HR 1.10, CI 1.11–1.15), male sex (HR 3.65, CI 1.21–11.04), and diabetes (HR 3.55, CI 1.35–9.38) predicted mortality, while OHS diagnosis did not. <bold>Conclusions:</bold> Overall, OHS patients had worse survival than controls. In OHS patients, age and non-adherence to PAP predicted survival. Among adherent OHS and controls, only age, male sex, and diabetes significantly predicted survival, while OHS diagnosis did not.