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Background. Uterine Mullerian adenosarcoma (UMA) is a rare and low- grade variant of mixed mesodermal tumor. UMA recurrences including heterologous sarcomatous elements are extremely rare, with only 3 cases reported thus far. Case. We present a case of UMA recurring as heterologous sarcoma three times in 8 months for 9.4 years after primary treatment. The number and grade of sarcomatous components increased with each recurrence eventually resulting in loss of the patient. Conclusion. The presence of multiple sarcomatous elements of high histological grade in the recurrent tumor following UMA may indicate an unfavorable prognosis with common recurrences and fatal outcome. All UMA patients carry risk of late recurrence, therefore, they should receive long- term follow- up.