IntroductionTreatment of urinary tract calculi in patients with Ehlers‐Danlos syndrome, a connective tissue disorder, has rarely been reported.Case presentationA 33‐year‐old woman with Ehlers‐Danlos syndrome sought evaluation of right‐sided abdominal pain from her family physician. Right‐sided hydronephrosis was noted and she was referred to our hospital for further evaluation and treatment. A ureteral calculus with a maximum diameter of 8 mm was demonstrated at the right ureterovesical junction. Transurethral lithotripsy was performed under general anesthesia without complications.ConclusionLithotripsy may be safely performed in patients with Ehlers‐Danlos syndrome.