Abstract Ehlers-Danlos syndrome (EDS) is a rare connective tissue disorder, usually characterized by the triad of hypermobility of the joints, hyperextensibility of the skin, and tissue fragility. Numerous gastrointestinal complications have been reported with this syndrome. However, spontaneous diaphragmatic rupture remains a very rare complication. We report a case of a 22-year-old woman with EDS who presented to the Emergency Department complaining of shortness of breath and left-sided chest pain. She was ultimately found to have a spontaneous diaphragmatic rupture after forceful emesis.