Background: Renal colic diagnosis is sometimes obtained only on clinical basis: acute flank pain with a history of nephrolithiasis.
Case report: This report shows up a severe clinical manifestation of vascular type Ehlers-Danlos syndrome in a young man, associated for the first time with incomplete distal renal tubular acidosis and a history of nephrolitiasis.
Conclusions: This clinical case suggests that it is necessary to perform at least ultrasonography to rule out life-threatening emergencies, which may be hidden by nephrolithiasis. It is the first time that renal tubular acidosis and nephrolitiasis represent the initial manifestation of vascular type Ehlers-Danlos syndrome.

Keywords: Acidosis, Renal Tubular, Ehlers-Danlos Syndrome