Rationale: Adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland with the highest malignant degree. ACC of the lacrimal gland is characterized by symptoms of <1 years duration. We present a 38-year-old male patient who complained of an enlarging mass in the left lacrimal fossa for almost 10 years previous to the diagnosis of ACC. Patient concerns: A 38-year-old male patient visited our ophthalmology clinic with a chief complaint of a mass in his left upper lid, which had enlarged significantly over the previous months. Diagnoses: Magnetic resonance imaging with intravenous Gadobutrol showed moderate and homogenous mass enhancement. Bone destruction is found. The periosteum is not eroded. The magnetic resonance imaging finding was supportive for malignancy. Histopathological examination of the specimen revealed solid tumor showing a cribriform pattern mixed small amount of basaloid cell proliferation. Therefore, the final diagnose was Adenoid cystic carcinoma of the lacrimal gland. Interventions: The treatment included en bloc resection of the mass and adjacent bone and radiotherapy. Outcomes: In 1 year follow-up after operation, there is no recurrence. Visual acuity is 30/30. The left eye shows limitation on abduction. Lessons: The present case demonstrates an unusual progression of ACC of the Lacrimal Gland.