Extrapulmonary small cell carcinomas (ESCCs) are poorly differentiated neuroendocrine tumors that are characterized by an aggressive course and poor survival rates. While these tumors can be found anywhere in the body, presentations of lesions in the orbit are exceedingly rare. We present the case of a 47-year-old man who presented with blurry vision, lacrimation, and tenderness of his right eye, as well as a small but palpable temporal mass. Upon workup, he was diagnosed with ESCC in the orbit as well as lesions in the liver and spine. He received systemic chemotherapy but unfortunately proceeded to have rapid spread of his disease and succumbed to this cancer only a year after presentation. This patient illustrates the importance of developing optimal treatment strategies, which have yet to be delineated, and especially the impact of newer immunotherapy agents remains to be seen.
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