Background: Urinary stone disease in cross-fused renal ectopia is rare and the aberrant anatomy poses challenges to treatment options. The available literature on treatment modalities remains limited. In this study, we present a case of ureteral stone in a cross-fused renal ectopia managed through retrograde approach. Case Presentation: We present a case of a 69-year-old woman with an obstructing ureteral stone in a cross-fused renal ectopia managed with ureteroscopic stone extraction. With the use of a ureteral access sheath, holmium laser, and Nitonol basket, the stone was fragmented and removed through retrograde access. The stone composition was 100% calcium oxalate monohydrate and her 24-hour urine collection was only significant for low volume. Conclusion: With special modifications and attention to the individual patient's anatomy, retrograde approach with the use of an access sheath is safe and effective for treatment of ureteral stones in patients with cross-fused renal ectopia.