Nickan Research Institute Journal of Parathyroid Disease 2345-6558 11 1 2023 01 01 Erdheim-Chester disease; a 31-year-old woman presented with bone pain and ophthalmologic involvement: a case report e11209 e11209 10.34172/jpd.2023.11209 EN Soheil Abdollahi Yeganeh https://orcid.org/0009-0008-1429-2098 Arash Dehghan https://orcid.org/0000-0001-9914-8862 Javaneh Jahanshahi https://orcid.org/0000-0002-0064-7408 Sepehr Shirouei https://orcid.org/0000-0002-8856-5668 Mahdi Arjipour https://orcid.org/0000-0002-0261-1492 Shiva Borzouei https://orcid.org/0000-0001-6826-9872 Journal Article 10.34172/jpd.2023.11209 2023 04 14 2023 08 10 Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder with no actual known incidence rate. It primarily affects multiple organs via increased expression of proinflammatory cytokines, eventually leading to histiocyte activation and infiltration at multiple sites. A 31-year-old virgin female presented with progressive generalized bone pain and blurred vision. She underwent a comprehensive clinical and paraclinical evaluation. Based on the final results, the diagnosis of ECD was established. We started treatment mainly using Interferon-α and corticosteroids, estrogen, levothyroxine, and desmopressin acetate. In adults with a mysterious chronic disease affecting multiple organs, we should always consider ECD as a differential diagnosis. Although ECD is still rare, the detection rate of this disease has increased significantly in recent decades. A comprehensive clinical and paraclinical evaluation is necessary to make a definitive diagnosis and determine the extent of the disease.