Background: The true incidence of upper tract cancers in the United States is not well defined, with cancers combined with kidney cancer and ureteral cancers grouped with rare urinary organ cancer. It is estimated to occur at 5% of urothelial carcinomas with a rough estimate of 3750 cases annually. Little data exists for non-urothelial variants of upper tract cancers. Methods: The primary objective of this retrospective review is to evaluate trends and differences between urothelial (UC) versus non-urothelial (nUC) histologies (squamous, sarcomatoid, small cell or neuroendocrine, adenocarcinoma) for upper tract cancers and compare the demographics, disease characteristics, treatment, incidence of stage and survival according to NCDB. Results: Data from diagnosis in year 2004 – 2017 were extracted from the NCDB. A total of 29743 urothelial and 561 non-urothelial cases for upper tract cancers were identified. More men were diagnosed with urothelial carcinoma, UC (62%) and non-urothelial carcinoma, nUC (58%) for non-urothelial carcinoma. The median age was similar for both groups, 73 years (UC) and 72 years (nUC). Majority were Caucasian at 92% (UC) and 91% (nUC) with incidence of 4% in the UC and 6% in the nUC cohorts for African-American patients. Primary surgery with nephroureterectomy occurred more frequently in the UC cohort (85%) compared to the nUC (58%). More patients were diagnosed with stage IV cancer based on the AJCC Clinicopathologic stage grouping in the nUC group (29%) compared to UC at 8.1%, but overall survival was not different for stage IV cancers with median OS of 8.57 mos (CI, 8.05-9.10) for UC vs 7 mos (CI, 5.62 – 9.26) nUC; logrank p = 0.283 although survival was significantly different for earlier stages of Stage 0 & 1 at 80.53 mos UC (CI, 78.13, 83.25) vs 28.98 mos nUC (CI, 20.3, 46.78), p < 0.001; and for stage II & III at median OS of 35.65 mos UC (CI, 33.38, 38.05) vs 15.75 mos nUC (CI, (11.17, 26.87) that is also significantly different though with lesser significance with longer follow-up. Conclusions: Upper tract urothelial cancers compared to non-urothelial cancers have similar poor outcomes upon diagnosis of metastatic disease though outcomes are with urothelial upper tract is better with earlier stage upon diagnosis compared to non-urothelial cancers, highlighting the importance of early diagnosis with perhaps curative intent treatment.