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Purpose The study aimed to summarize all published cases of intrinsic brainstem epidermoid cysts in a timeline to highlight the specific characteristics and individualize the disease, in addition to discussing the best treatment used. Methods The scientific literature on pediatric cases of intrinsic epidermoid cysts of the brainstem was analysed. We present the case of a1,5-year-old male with incidental apresentation, who was treated with gross total resection. We summarize all previously published cases to individualize the disease. Results We identified 21 patients, including 10 boys and 11 girls, with a mean age of 4.85 (1–15) years at the time of surgery. The most frequent symptoms were cranial nerve palsy (71.4%), pyramidal tract deficit (57.14%) and headache (52.38%). Among the affected cranial nerves, VII was the most frequent, reported in 10 patients. Conclusion Brainstem epidermoid cysts are extremely rare pathologies with relevant age involvement in young children. The treatment objective should be the maximum resection of the |
