enchondroma of the skull base in a case of ollier’s syndrome

ollier’s syndrome, a variant of multiple enchondromatosis, is a rare disease with an estimated prevalence of 1/100,000, characterized by multiple enchondromas, asymmetrically involving small bones of the hands and feet, especially the proximal phalanges. intracranial enchondromas, such as those arising from the skull base are extremely rare. herein, we report a 25-year-old female, known case of ollier’s disease, presenting with right eyelid ptosis and visual disturbance. brain mri revealed a skull base tumour suspicious to enchondroma followed by trans-sphenoidal resection. histologic examination of the excisional biopsy sample confirmed the diagnosis of enchondroma.   how to cite this article: sabeti s, yousefi f, pourabdollah toutkaboni m. enchondroma of the skull base in a case of ollier’s syndrome. iran j pathol. 2015;10(3):237-42.