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An accessory cavitated uterine mass (ACUM) is a very rare obstructive genital malformation characterized by pelvic pain and severe dysmenorrhea. It is easily mistaken for other obstructive genital malformations in women, such as cystic uterine adenomyosis or cystic degeneration of uterine fibroids. This case report describes a 30-year-old patient with a huge uterine cornual mass. Successful resection was performed by surgical excision, and the lesion was diagnosed as an ACUM. Given the rarity of a giant ACUM, this report also includes a brief review of the relevant literature.