Introduction: Clear Cell Myomelanocytic Tumor (CCMMT) of ligamentum teres hepatis is a pathological classification of Perivascular Epithelioid Cell tumor (PEComa), which is rare clinically and easy to misdiagnose. Objective: To report a case of a rare type of PEComa located in the ligamentum teres hepatis. Case Report: A 22-year-old Asian female was diagnosed with abdominal mass during physical examination in September 2018, and was admitted to the general surgery department of our hospital that month. She was diagnosed with abdominal mass, the nature of which remains to be determined is: teratoma, pheochromocytoma or ganglioma. At the time of admission, the patient had no symptoms or signs, and no other medical history. The patient was diagnosed with an abdominal mass by abdominal plain scan and enhanced CT, whose nature was to be determined: pheochromocytoma, paragangliomas or other mesenchymal tumors, or giant lymph node hyperplasia. The patient underwent abdominal mass resection and appendectomy without incident, without any complications at discharge, and there was no significant difference in follow-up. Conclusions: The clinical data, imaging features and pathological features of one patient diagnosed with CCMMT in our hospital were retrospectively analyzed, and the literature was reviewed in combination with the research progress of CCMMT, in order to improve the understanding and diagnostic accuracy of this disease.