Background Primary lymphoma of the cavernous sinus is a rare form of extranodal non-Hodgkin lymphoma, of which very few cases have been reported in the published literature. This report presents the MRI findings with apparent diffusion coefficient (ADC) value in an exceedingly rare primary marginal zone B-cell lymphoma (MZBCL) of the cavernous sinus. Case presentation The case in this study is a 59-year-old immunocompetent male patient with a 2-month history of right ptosis and blurred vision. Right third cranial nerve palsy and binocular diplopia were observed upon neurological examination. Preoperative brain CT showed an extra-axial enhancing mass lesion in the right cavernous sinus. On MRI, ipsilateral internal carotid arterial encasement was noted without causing stenosis of the vessel. Isointense signal on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and diffusion restriction were also observed. The mean ADC value of the tumor is 0.64 x 10(-3) mm(2)/s (b value = 1000 s/mm(2)). Subtotal resection of the tumor was performed, and improvement of clinical symptoms were observed. The pathologic diagnosis of MZBCL was established by immunohistochemical examinations. Conclusions Primary MZBCL of the cavernous sinus is exceedingly rare, and preoperative confirmation poses a major challenge with CT and conventional MRI only. In this case, preoperative quantitative ADC value is shown to offer valuable additional information in the diagnostic process.