<p>Epidermoid cysts comprise about 1% of intracranial tumors, they are benign and extra-axial, arising from ectopic ectodermal tissue. Pineal region localization is rare. Surgical treatment can be challenging due to critical structures encountered along surgical corridors. We aimed to synthesize the literature compiled on this disease and present an illustrative case. We performed a scoping review according to PRISMA-Sc guidelines. Studies describing pineal region epidermoid cysts reporting clinical, surgical, or radiological data were included. A total of 33 patients from 31 studies were included in this review. The publication year ranged from 1990 to 2024. The mean age of patients was 30.5 years. The mean follow-up period was 18.9 months. On MRI, the cysts most commonly followed CSF intensity, except on diffusion imaging on which there was restriction for all cases. Hydrocephalus was present in 85% of patients, 43% of which underwent permanent CSF diversion. The most common surgical approach was the supracerebellar infratentorial approach. There was no post-operative mortality reported. Epidermoid cysts located in the pineal region are very rare and their diagnosis relies on MRI characteristics, as they typically follow CSF intensity on T1 and T2 weighted sequences, with diffusion restriction patterns, however without contrast enhancement. Although these are benign lesions, their location requires thoughtful surgical planning and high-level microsurgical expertise given the surrounding critical structures. The selection of the ideal approach is dependent on the patient’s anatomy and the characteristics of the lesion.</p>