The "Tailgut cyst" is a unique dysontogenetic entity, whose source of origin is marked by the postnatal persistence of the primitive intestine in the presacral space. Predominantly female in distribution, this generally benign congenital tumor can experience malignant degenerative changes to varying degrees. Given its rarity, its location and its non-specific clinical footprint, the condition most often constitutes a diagnostic dilemma. The management of the pathology is surgical excision, considered as the curative treatment also allowing a final histopathological diagnosis. In this report, we present the case of a woman with a history of Tailgut cyst diagnosed by imaging following exploration for pelvic discomfort and which was successfully managed and pathologically confirmed to be benign after complete surgical resection. We are reviewing the literature to improve understanding and increase awareness of this exceptional clinical scenario in our workflow
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