Introduction. Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare malignant neuroecto dermal nasal tumor with distinctive clinical, histopathological, radiological, and molecular features. It arises from olfactory receptors in the nasal mucosa or the cribriform plate of the ethmoid bone. It is generally slow-growing; fast-growing tumors may lead to widespread metastasis. Here, we present an atypical case of aggressive esthesioneuroblastoma treated with a multimodality approach. 

Case description. A 28-year male, presented with a painful swelling over the right cheek lasting for 5 months. The patient underwent surgery, and histopathology of the surgical specimen revealed a small round blue cell tumor with widespread positivity for synaptophysin and CD 56. The histopathological appearance and immunohistochemical profile of the biopsy tissue confirmed esthesioneuroblastoma. Owing to subtotal resection, the patient received adjuvant radical radiotherapy to the local site and lymph nodes. Three months post-radiotherapy, a CECT scan showed a partial response, so the patient received intravenous chemotherapy. However, the patient had local disease progression; establishing the aggressivness of esthesioneuroblastoma in our patient. The patient is alive with residual stable disease after 2.5 years from the initial diagnosis and is follow up. 

Conclusion. Esthesioneuroblastomas are uncommon tumors and owing to their slow-growing nature, the patient may neglect them. There is a wide spectrum of clinical presentations and outcomes in such patients, and as the literature on esthesioneuroblastoma is scarce due to its rarity, this case report seeks to contribute to a better understanding of such uncommon malignancy in terms of its clinical presentation, behavior, and outcomes.