BACKGROUND: Laparoscopic management of remnant uterine structures for patients with persistent mullerian duct syndrome (PMDS) and bilateral intra-abdominal testes include supracervical hysterectomy or splitting of the uterine structure to facilitate orchiopexy. A laparoscopic uterine-sparing approach, however, has not been described in the literature. METHODS: We present a case of a 10-year-old male with PMDS who underwent laparoscopic two-step Fowler-Stephens orchiopexy (FSO) with uterine preservation. Diagnostic laparoscopy revealed bilateral intra-abdominal testes, a robust right vas deferens but diminutive left vas deferens, and a rudimentary uterine structure posterior to the bladder. At the time of the second-stage FSO, the decision was made to preserve the uterine structure to keep all future fertility options viable. A more extensive dissection was undertaken on the left side to gain adequate length for both testes to reach the scrotum and give the best chance for survival to the right testis with its accompanying robust vas deferens. CONCLUSION: Our case highlights a laparoscopic approach to a challenging problem in pediatric urology. If uterine preservation is preferred, a laparoscopic two-step FSO with uterine preservation is technically feasible and should be a consideration for patients with PMDS and intra-abdominal testes.