We report a case of primary central nervous system (CNS) malignant lymphoma of the central nervous system originating from the cerebellum and growing along the lower cranial nerves. A 67-year-old woman presented with hoarseness, vertigo, nausea, and vomiting. Gd-DTPA enhanced MRI showed a homogeneous enhanced mass lesion extending from the cerebellum to the medulla oblongata around the jugular foramen on the right side. Although pre- and intra-operative diagnosis had been schwannoma, histopathological examination revealed a B-cell, diffuse malignant lymphoma. The growth pattern of malignant lymphoma in the present case, which extended extra-axially, is considered to be rare. We discuss here the growth patterns and difficulties of diagnosis of primary CNS malignant lymphoma in this area.