Central nervous system (CNS) metastasis from T cell lymphoma represents a rare clinical scenario. The subtypes of T cell lymphoma most frequently associated with CNS involvement include peripheral T cell lymphoma (PTCL) and anaplastic large cell lymphoma (ALCL). Neurologic symptoms of secondary CNS involvement tend to be acute or subacute. Diagnostic measures encompass neurologic examination, gadolinium-enhanced MRI of the entire neuraxis, cerebrospinal fluid (CSF) analysis, and, potentially, brain biopsy. Due to their rarity, CNS metastases from T cell lymphoma pose a diagnostic challenge and must be discriminated from other diseases such as infectious, autoimmune, and other neoplastic causes. Treatment modalities comprise systemic chemotherapy, intrathecal chemotherapy, and radiation therapy. Despite numerous treatment options, the prognosis for patients with CNS metastases from T cell lymphoma is generally poor. This case report describes a patient with biopsy-proven CNS metastases from peripheral T cell lymphoma. The narrative literature review aims to improve understanding of the clinical characteristics, diagnostic modalities, treatment options, and prognosis of patients with CNS metastases from peripheral T cell lymphoma.