Introduction Paragangliomas (PGLs) are frequently reported around the abdominal aorta; however, are extremely rare near the urachus. Case presentation A 78‐year‐old woman was referred to the urology department of our hospital for further examination and treatment of a 1.2‐cm tumor in the lower abdominal wall, a tumor excision was then performed. On immunohistochemical staining, the tumor and supporting cells were positive for chromogranin A and the S 100 protein, respectively, and were diagnosed as PGL. The PGL was thought to be derived from chromaffin cells that migrated to the wall of the urachus during embryonic life and remained even after the wall regressed. Conclusion We report a case of PGL near the urachus that can be explained by the distribution of the sympathetic network around the midline of the lower abdominal wall during embryonic development. Therefore, PGL should be considered in the differential diagnosis of periurachal tumors.
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