RESUMO
Secondary metastasis of rhabdomyosarcoma (RMS) to the orbit from a distant primary site is extremely rare in adults. In this article, we describe the case of a 24-year-old male presenting with proptosis, diplopia, and headaches concurrently with a histologically confirmed diagnosis of PAX3-FOXO1 positive paraspinal alveolar rhabdomyosarcoma. An orbital MRI revealed a fusiform mass arising from the inferior rectrus, displaying necrotic and irregular morphology consistent with malignancy. The patient is currently undergoing intensive chemotherapy. The objective of this case report is to highlight the rarity of an extraocular metastasis of RMS in an adult patient, alongside the importance of considering metastatic disease in a patient with fulminant unilateral proptosis.