The ethics of invasive mechanical ventilation for children with the neurodegenerative disease Spinal Muscular Atrophy Type I (SMA I) is highly debated, and wide variability in clinical outcomes exists internationally. We conducted this international survey to identify physician characteristics associated with recommendation for tracheostomy and ventilation for SMA I. A cross-sectional online survey was distributed to 1,772 pediatric pulmonologists and pediatric intensivists from online membership directories of American Thoracic Society, American College of Chest Physicians, and European Respiratory Society. Questions explored physician demographics, attitudes and experience with SMA and end-of-life care, knowledge of consensus guidelines, and recommendations for respiratory care of SMA I. A logistic regression model assessed the independent effects of physician variables on the recommendation for invasive ventilation for SMA I. A total of 367 (21%) physicians completed the survey; 82% were pediatric pulmonologists; and 16% pediatric intensivists. Seventy percent of respondents were from the U.S. Fifty percent of physicians were aware of SMA consensus guidelines. Physicians from Commonwealth countries (U.K., Canada, Australia, etc.) were less likely to recommend tracheostomy/ventilation than U.S. physicians (7% vs. 25%, P = 0.005). Logistic regression modeling identified years of experience, pediatric pulmonology specialty, agreement with a pro-life statement, and recommendation for non-invasive ventilation as predictive of recommendation for long-term invasive ventilation for SMA I. In the largest international survey on this topic, we identified regional differences in physician recommendation for invasive ventilation for children with SMA I. Our data demonstrate a need for increased awareness of consensus guidelines and further dialog about the physician role in variability of care for children with SMA I.