Although adenoid cystic carcinoma (ACC) of the lacrimal gland is a rarely encountered orbital tumor, it invades intracranially more frequently than carcinomas of other glands in the head and neck. A 52-year-old man underwent orbital exenteration and resection of intracranially extended tumor via a fronto-orbito-zygomatic approach in combination with a transfacial approach. Histopathologically, the tumor showed perineural, vascular, and lymphatic invasion. Additionally, he received radiotherapy (60 Gy) and adjuvant systemic cisplatin and 5-fluorouracil chemotherapy due to residual tumor in the orbit and systemic metastases (lung, ribs, and spines). He was free of progression and recurrence at 6 months after treatment. The authors report a case of skull base invasion by an ACC of the lacrimal gland to remind neurosurgeons planning intervention that this disease shows a tendency to invade intracranially.