We report two patients with diffuse large B-cell lymphoma with isolated oculomotor nerve palsy. This was the initial manifestation of lymphoma in one patient but in a second, the palsy appeared as a part of a generalized recurrence of lymphoma. In addition, we reviewed the clinical findings of 12 previously reported patients. Isolated oculomotor nerve palsy was most frequently associated with the large B-cell lymphoma cell type, and was not frequently associated with periorbital pain. The pupil was spared in half the patients irrespective of infiltration of the oculomotor nerve. MRI and cerebrospinal fluid cytological examinations are helpful in determining invasion of lymphoma to the oculomotor nerve, cavernous sinus, and surrounding leptomeninges.