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Purpose: Urinary incontinence (UI) affects an estimated 53% of women and negatively affects quality of life. Stress UI (SUI), involuntary leakage of urine with activities that increase intra-abdominal pressure, is the most prevalent subtype. Chronic lung disease is a suggested risk factor based on studies in obstructive disorders reporting association with cough. SUI has not been examined in interstitial lung disease (ILD) where cough is less prevalent. This study investigates SUI in lymphangioleiomyomatosis (LAM)– a rare, progressive ILD typically diagnosed in otherwise healthy women of childbearing age. Methods: In this cross-sectional study, a questionnaire assessing urinary and respiratory symptoms, lung function, fatigue, UI reporting, and health status was completed by 167 women with LAM plus 218 healthy controls matched for major SUI risk factors (CON). Analysis was performed with Welch t-test (continuous data) and chi-square and Mann–Whitney U tests (ordinal data), and risk differences using linear regression determined risk factors for SUI. Results: The prevalence of SUI was higher in LAM (72%) compared with matched CON (48%). LAM with SUI had more dyspnea, phlegm production, and fatigue-related symptoms, but not more cough or lung impairment. Less than a third of LAM with SUI reported symptoms to medical providers, and prescribed treatments were commonly inconsistent with current recommendations. Conclusions: A higher prevalence and underreporting of SUI in women with LAM suggests need for targeted SUI screening and management to address a potentially overlooked complication of the disease with known impact on QoL. Future research should investigate how respiratory sequelae may contribute to greater SUI in LAM and other chronic lung diseases.