Abstract
Adenoid cystic carcinoma (ACC) in the lacrimal gland is a very rare disease with poor overall prognosis. Its primary treatment is surgical excision, including orbital exenteration and radical orbitectomy, which is combined with radiotherapy and chemotherapy. Age, histopathologic type, bone invasion, and tumor extent are known factors that affect the prognosis of ACC. Furthermore, perineural invasion is highly associated with local tumor recurrence and tumor base invasion. Here, we report a rare case of ACC in the lacrimal gland with superior sagittal sinus invasion that repeatedly recurred after the surgical excision.