Nephrogenic adenoma (NA) is an unusual benign epithelial tumor in the genitourinary tract. Here we report a fibromyxoid nephrogenic adenoma in a 37-year-old female presenting with over 10-year slow-growing renal pelvic mass that was diagnosed with bland spindle cell lesion in multiple previous biopsies. This is the first reported case of pure fibromyxoid NA in renal pelvis with close comparison and correlation of biopsy and resection findings over a 10-year span. This will enhance awareness of pathologists to consider this unusual entity when examining spindle cell lesions in this setting, and prevent misdiagnosis and overtreatment of a typically benign process.