To contrast surgical outcomes of Horseshoe Kidney (HSK) patients with localized renal masses suspected of cancer with nonfused nonectopic kidney patients, emphasizing safe surgical practices for HSKs. The study examined solid tumors from the Mayo Clinic Nephrectomy registry between 1971 and 2021. Each HSK case was matched to three non-HSK patients based on various factors. The outcomes measured included complications within 30days of surgery, change in estimated glomerular filtration rate, and overall, cancer-specific, and metastasis-free survival rates. Thirty of the 34 HSKs had malignant tumors compared with 90 of the 102 patients in the nonfused nonectopic referent cohort. Accessory isthmus arteries were present in 93% of HSK cases, with 43% exhibiting multiple arteries and 7% with 6 or more arteries. Estimated blood loss and surgery duration were significantly higher in HSKs (900 vs 300mL, P=.004; 246 vs 163minutes, P<.001, respectively). The HSK group demonstrated an overall complication rate of 26% (vs 17% in referents, P=.2) and a median change in estimated glomerular filtration rate at 3months of -8.5 (vs -8.1 in referents, P=.8). At 5-year follow-up, survival rates for HSK patients were 72%, 91%, and 69% for overall, cancer-specific, and metastasis-free survival, respectively. The corresponding rates were 79%, 86%, and 77%, respectively, for matched referent patients (P>.05). HSK tumor management is technically challenging with higher blood loss; however, the data demonstrate comparable outcomes for patients with HSK tumors, including complications and survival, to those without HSKs in experienced centers.