BACKGROUND Rosai-Dorfman-Destombes disease is a rare and heterogeneous entity that has been associated with autoimmune, hereditary, and malignant diseases. There is controversy about its etiopathogenesis, clinical course, and therapeutic management. OBSERVATIONS The authors report a case of a 61-year-old man with a history of progressive headache without any other symptoms. Magnetic resonance imaging of the brain revealed multiple irregular lesions with an initial diagnostic impression of meningiomatosis. An excisional biopsy was performed, and the pathology report stated the finding was Rosai-Dorfman-Destombes disease. LESSONS The uniqueness of this case is its rarity. The isolated intracranial location presents many diagnostic and therapeutic challenges, with radiological and clinical characteristics similar to those of other central nervous system tumors. There is currently no clear evidence of the pathogenesis and therapeutic management of this condition. Follow-up of these patients will help elucidate the natural history of this condition and the benefits of various treatment modalities.

Background: Myoepithelial tumors have been widely described as a rare form of salivary gland neoplasm, although currently soft-tissue phenotypes have also been identified. These are tumors composed entirely of myoepithelial cells that exhibit a dual epithelial and smooth muscle phenotype. The occurrence of myoepithelial tumors within the central nervous system is also extremely rare, with only a few cases reported. Treatment options include surgical resection, chemotherapy, radiotherapy, or a combination of these approaches. Case Description: The authors present a case of soft-tissue myoepithelial carcinoma with an unusual brain metastasis, rarely described in the literature. The purpose of this article is to present an update on the diagnosis and treatment of this pathology when affecting the central nervous system, through the review of the current evidence. Conclusion: However, despite complete surgical resection, there is about a significative high rate of local recurrence and metastasis. Careful patient follow-up and staging is essential for better characterization and understanding of this tumor’s behavior.