Objective: To describe two rare cases of multiple extrauterine adenomyomas, their clinical management, and their response to surgical and hormone therapy with GnRH agonist. Design: Case report and review of the English-language scientific literature. Setting: Cases presented in a tertiary health care academic institution. Patient(s): Two patients without urogenital malformations diagnosed with extrauterine adenomyoma, which is a benign tumor composed of smooth muscle and endometrium, typically originating within the uterus. Intervention(s): Surgical radical excision of adenomyomas followed by a long-term hormone therapy with GnRH agonist. Main Outcome Measure(s): Anatomical and clinical outcomes. Result(s): Surgical treatment followed by long-term GnRH agonist therapy appeared effective in keeping the disease stable. After a long-term follow-up of 10 and 4 years, the two patients are still asymptomatic and stable. Conclusion(s): Only 19 other cases of extrauterine adenomyomas are reported in the English-language scientific literature, and no cases of multiple masses are described until now. It is probable that these extrauterine adenomyomas arose from a metaplastic transformation of the subcelomic mesenchyme. Long-term GnRH agonist therapy after surgery appeared effective in keeping the disease stable.