BACKGROUND Angiolipoma is a benign mesenchymal tumor that usually occurs in the sub-skin tissue of limbs and the trunk. However, intracranial angiolipomas are rarely reported, and most of intracranial angiolipomas reported were found in the sellar and parasellar areas. We report the case of a 23-year-old man presenting with ataxia and bilateral upper-limb tremor due to a pineal region angiolipoma resected endoscopically by supracerebellar infratentorial approach. We also reviewed the literature to explore the etiology, locations, features, and surgical outcomes of intracranial angiolipomas. CASE REPORT A 23-year-old man with ataxia and tremor of both upper limbs was transferred to our hospital. Cranial magnetic resonance imaging (MRI) revealed an obviously enhancing pineal region lesion (20×26×29 mm) with mass effects on the quadrigeminal bodies of the midbrain. A purely endoscopic supracerebellar infratentorial approach craniotomy was performed, achieving complete resection of the tumor. Histologically, the tumor was composed of mature adipose cells and blood vessels, consistent with the pathological diagnosis of angiolipoma. During the clinical follow-up of 3 months, the patient gradually recovered completely without any neurological dysfunction. Postoperative MR further verified total tumor resection without signs of recurrence or brain stem and cerebellar infarction. CONCLUSIONS This case provides intraoperative and histological evidence of an angiolipoma in the pineal region. Additionally, our case demonstrates successful surgery for intracranial angiolipoma removal in the pineal region, highlighting purely endoscopic surgery as a viable treatment option for this condition.