Background: Erdeheim-Chester disease is an uncommon histiocytosis. Fifty-nine cases have been reported in the literature. Bone lesions are usually inaugural followed by multiorgan involvement combining bone disease, orbital infiltration, diabetes insipidis and retroperitoneal infiltration.
Case report: A 53-year-old man had Erdheim-Chester disease which progressed over 11 years. The patient developed extrinsic obstruction of the upper urinary tract. This unusual complication of Erdheim-Chester disease raised a difficult therapeutic problem as percutaneous drainage was impossible. The patient was treated with an endoprothesis allowing urine derivation. Surgical ureterolysis was avoided.
Discussion: Data in the literature favor use of minimally invasive endourological treatment for patients with urinary tract complications of Erdheim-Chester disease.