A 53-year-old woman was admitted to the hospital one year after the onset of her complaints. She suffered from epiphora and swelling in the lacrimal sac region. Biopsy revealed islets of classical basal cell carcinoma with peripheral formation of palisades within a hyperemic stroma. During surgery, the lacrimal drainage system and neighboring bony structures were completely excised. No local recurrences were detected on orbital tomography performed 6 and 15 months postoperatively. As far as the authors know, this is the first case of basal cell carcinoma in the lacrimal sac reported in the literature.