A 72-year-old female complained of acute pain on left eye movement followed by progressive exophthalmos. Neuroimaging revealed a large well-demarcated lesion consisting of solid and cystic parts, as well as bone destruction and hemorrhage, within the left orbital cavity. The preoperative diagnosis was pleomorphic adenoma with or without malignant transformation, or cavernous angioma. En bloc excision including adjacent tissues was planned to resolve the progressive symptoms and to obtain a histological diagnosis. The transcranial route was chosen since tumor invasion to the cranial base was possible. The histological diagnosis was pleomorphic adenoma. Pathological and preoperative radiological examinations indicated that repeated intratumoral hemorrhage had caused the orbital bone destruction and acute orbital pain. Neoplasms should be differentiated from a wide spectrum of other possible pathologies. Accurate clinical diagnosis of neoplasm in the orbital cavity is important for correct therapeutic management. Malignancy is generally suspected if painful and progressive signs and symptoms are associated with an orbital mass lesion. The present case suggests that pleomorphic adenoma should also be considered in the differential diagnosis. The therapeutic strategy for lacrimal gland tumors remains controversial, so a flexible management approach is required.