RESUMO
A case of a 6-year-old boy presenting with epiphora, medial canthal swelling, and lymphadenopathy is reported. Biopsy revealed a poorly differentiated squamous cell carcinoma. The child underwent neoadjuvant chemotherapy and showed tumor progression after a remarkable initial response. This case highlights the atypical age of presentation of potentially fatal lacrimal sac malignancy. Further, it emphasizes the importance of knowing signs of lacrimal sac malignancy among ophthalmologists treating childhood epiphora where inflammatory etiologies are most common. A high index suspicion, thorough clinical examination, and knowledge of the signs of malignancy may avoid delay in diagnosis of this rare but life-threatening malignancy. [J Pediatr Ophthalmol Strabismus. 2019;56:e8-e11.].