Acute myeloid leukemia (AML) is a disorder of the myeloid cell line that can manifest infrequently as a granulocytic sarcoma with infiltration into bone and soft tissue. Consequently, cranial nerve neuropathy due to AML infiltration can result in variable neurological deficits, including facial nerve palsy. Here, we present the case of a patient presenting with unilateral facial nerve palsy with evidence of AML in cerebrospinal fluid (CSF) cytology and bilateral opacification of the mastoid air cells suggestive of AML infiltration into the mastoid process. Patient demonstrated improvement of facial palsy after administration of intrathecal chemotherapy without need for surgical intervention. We further examine known cases reported to date on the use of chemotherapy and surgical intervention in management of facial nerve palsy as a consequence of AML infiltration of the mastoid bone. Notably, there appears to be a correlation between mastoid bone infiltration seen on imaging and facial nerve palsy in patients with known history of AML that may be treated without need for surgical intervention or biopsy.