HighlightsAngiomyxomas are rare benign neoplasms tumors arising from the soft tissues of the perineum and pelvis.Although benign, they are notorious for their locally infiltrative nature and their propensity to recur.To the best of our knowledge, we report the first case of ureteral angiomyxoma in the literature presenting with hematuria.If an angiomyxoma were to be considered in our differential diagnosis, a ureteral biopsy could have led to an organ-sparing surgery.
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