Abstract
RATIONALE: Persistent Müllerian duct syndrome (PMDS) is rare form of male pseudohermaphroditism characterized by the presence of uterus and fallopian tubes with normal external genitalia and secondary sexual characteristics. Transverse testicular ectopia (TTE) is also a rare form of testicular ectopia that may be associated with PMDS. PATIENT CONCERNS: We present a 2-year-old boy who presented with bilateral non-palpable testes with left inguinal mass. DIAGNOSIS: TTE with PMDS. INTERVENTIONS: On exploration, both testes were present in the left inguinal region. Uterus and fallopian tubes were located between the testes. A hysterectomy was perfomed with resection of the underdeveloped fallopian tubes. Bilateral orchiopexy was performed by placing both gonads into subdartos pouches in each scrotum with transseptal approach. OUTCOMES: Both testes were palpable in both the scrotum at 1-year postoperative follow-up and we are planning a regular follow-up. LESSONS: In case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Müllerian duct should be needed. A long-term postoperative follow-up is necessary for assessment of malignant transformation and infertility.