Background: Schwannomas are generally benign neoplasms arising from the nerve sheath. Presacral schwannomas are very rare entities and difficult to diagnose, representing less than 15% of all retrorectal space tumors. Benign schwannoma sometimes displays degenerative changes, such as cyst formation, calcification, hemorrhage, and hyalinization. Usually these degenerations are partially seen in the tumors. Objective: To point out that presacral schwannoma can display markedly multilocular cystic degeneration. Case Report: We present this unique case of a 24-year-old man diagnosed with an unusually large pure multilocular cystic schwannoma, which is revealed by digestive, urinary, and nonspecific neurological symptoms. The patient was successfully treated with radical surgery via an anterior approach leading to the recovery of symptoms. Discussion and Conclusion: This tumor was unusual in its totally multicystic appearance and its resemblance to a wide spectrum of lesions that can occur in the pre-sacral space, such as hydatid and developmental cysts. Preoperative diagnosis is essential to prevent major neurological deficits during surgical intervention.
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