Background: Nephrogenic adenoma is a benign lesion found in the genitourinary tract, often at sites of prior inflammation, and is characterized by tubular, papillary, or tubulopapillary structures. It is thought to arise from distal migration and implantation of renal tubular cells into the renal pelvis, ureter, bladder, or urethra. These tumors often resemble malignant neoplasms. Morphologic variants include small tubules, signet ring-like pattern, papillary formations, flat pattern, and vessel-like structures. A fibromyxoid variant was first described in 2007. Here, we present the first known cases of fibromyxoid nephrogenic adenoma of the ureter. Case Presentations: A 79-year-old white man presented with asymptomatic right hydroureteronephrosis to the level of the mid-ureter with associated right ureteral wall thickening found on surveillance CT scan for lymphoma. A 59-year-old white man presented with a right ureteral stricture after ureteroscopic ureteral injury and underwent effective robotic ureteroureterostomy. Pathology analysis in both cases revealed fibromyxoid nephrogenic adenoma. Conclusion: Fibromyxoid nephrogenic adenoma may occur in the ureter. Knowledge of this rare tumor is important for urologists and pathologists to prevent misdiagnosis and overtreatment of a typically benign process.