Nephrogenic adenoma is a rare and benign tumour of the urinary tract thought to be caused by metaplastic change of native urothelial tissue. The majority of cases arise in the bladder, with very few cases affecting the ureter reported in the literature. Herein, we describe the presentation, diagnostic challenges, and the eventual management of a nephrogenic adenoma of the ureter by robot-assisted laparoscopic nephroureterectomy. Urologists and pathologists should be aware of the potential diagnostic and management pitfalls associated with this rare tumour, as well as the sparsity of evidence with respect to follow-up.