Primary cutaneous extranodal natural killer/T-cell lymphoma, nasal type (ENKL-NT) is a relatively rare disease associated with aggressive tumor-cell behavior and poor prognosis. Progress in immunohistochemistry has improved the identification of ENKL-NT. The present case study reported on a 64-year-old female patient presenting with several red nodular lesions on the neck developed over four months. Cutaneous biopsy revealed these cells were positive for CD3, CD56, CD5, CD8 and negative for CD2, CD34, CD7, CD20 and Granzyme B. A computed tomography scan and bone marrow biopsy did not show any abnormalities and a diagnosis of primary cutaneous ENKL-NT was made. After treatment with chemotherapy regimens comprising cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP), followed by high-dose treatment with methotrexate, dexamethasone, ifosfamide, etoposide and L-asparaginasum (SMILE), the patient succumbed to the disease. The present study exemplified that immunohistochemical analysis as well as the recognition of atypical lymphoid cells showing angiocentricity is crucial for the correct diagnosis of ENKL-NT. The prognosis of primary cutaneous ENKL-NT remains poor. As the CHOP regimen is not completely effective, high-dose chemotherapy, such as the SMILE regimen, is required.