Introduction. Both benign and malignant tumors can develop in the retrorectal or presacral space. Most patients are asymptomatic and, when they do present symptoms, they are nonspecific. Among retrorectal tumors, the group of neurogenic origin stand out, where Schwannoma is the most frequent one. Clinical case. A 32-year-old woman with a retrorectal tumor, which present with imprecise symptoms, diagnosed during a surgical procedure due to a uterine myoma, which finally turned out to be a Schawnnoma. Conclusion. Computed tomography and magnetic resonance imaging are important for diagnosis and for establishing the level of the lesion in relation to the sacrum. The cornerstone of treatment is surgical resection. The approach can be anterior (abdominal), posterior (perineal, transsacral or parasacrococcygeal), or combined, according to its location at the S4 level.