Malignant fibrous histocytoma (MFH) of the maxillary sinus is believed to be a rare form of soft tissue sarcoma with a low frequency of distant metastasis. In this study, we provide a histological documentation of the hematogenous spread of MFH to the brain and report a case of maxillary sinus MFH with unusual metastasis to the brain. To our knowledge, this is the first case of a direct histological diagnosis of maxillary sinus MFH with brain metastasis via a hematogenous route.