繆勒氏導管殘跡症候群合併睪丸對側異位，出現於一正常外貌表徵的男性(46xy)，其臨床表現是痛且不可復原的右側鼠蹊部種塊及左側隱睪，在嵌塞性鼠蹊部疝氣的診斷下進行手術，手術中發現疝氣囊中存在著子宮，輸卵管及兩側睪丸。繆勒氏導管殘跡症候群是非常罕見，且只被診斷於手術當中，在此我們參考及整理相關文獻，對此一見病例做一簡單報告。

A case of persistent Mullerian duct syndrome with crossed testicular ectopia in a phenotypically normal male with karyotype 46 XY is presented. The clinical presentation was an irreducible, painful right inguinal mass and a left undescended testis. Under the impression of incarcerated inguinal hernia, the inguinal lesion was operated upon and found to be a uterus and a Fallopian tube. Persistent Mullerian duct syndrome is ery rare, and is usually found only during surgery.