Multiple endocrine neoplasia (MEN) is defined by the presence of at least two functionally unrelated endocrine gland tumours in the same subject. There are three types of MEN. MEN type 1 (or Wermer syndrome) is an autosomal dominant cancer syndrome. In the light of a case report, the authors recall the characteristics of MEN type 1 responsible for severe and recurrent urolithiasis and emphasize the need for systematic aetiological work-up for all patients presenting a first episode of urolithiasis according to the guidelines of the Association Française d'Urologie Stone Committee (CLAFU).