%0 Case Reports %T Aggressive angiomyxoma: The first case report in skull. %A Cao Z %A Miao L %A Liu M %A Liu W %A Zhang H %A Liu X %A Wang J %A Wang X %A Cao Z %A Miao L %A Liu M %A Liu W %A Zhang H %A Liu X %A Wang J %A Wang X %J Front Surg %V 9 %N 0 %D 2022 %M 36061044 %F 2.568 %R 10.3389/fsurg.2022.985739 %X Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor primarily growing in the soft tissue of the pelvis and perineum in women of reproductive age. It is a benign tumor that still has a probability of being accompanied by localized invasion. Although negative margins of resection are difficult to achieve due to the invasive nature of the tumor and the lack of a well-defined capsule, the first line of treatment for AAM is surgery. The diagnosis of AAM is difficult to make due to a lack of specific manifestations and specific tumor markers. In this study, we reported a case of aggressive angiomyxoma in a 2-year-old girl that rarely develops in the skull with craniocerebral compression. The patient initially had a mass on her head that attracted the attention of her family, and then she began to have episodic headaches. Surgery was performed after hospitalization, and the tumor recurred 1 year after the operation, around the originally affected skull.