Little is known about the diagnostic and prognostic markers of upper urinary tract urothelial cancer (UTUC) because of its rarity. ANXA10, a calcium dependent phospholipid binding protein, is differentially expressed in several cancers. It has been reported that ANXA10 expression is a urinary mRNA biomarker for detection of bladder urothelial cancer (BUC), and loss of ANXA10 expression is associated with BUC progression. To clarify the significance of ANXA10 in UTUC, we studied ANXA10 expression with immunohistochemistry (IHC). We analyzed ANXA10 by IHC in 117 respective cases of UTUC treated by radical nephroureterectomy without neoadjuvant chemotherapy. ANXA10 positivity (ANXA10+) was defined as >10 % of nuclear staining. ANXA10 expression was weak in normal urothelium of upper tract and was positive in 39/117 (33%) of UTUC. ANXA10+ was more frequently in tumors with pure UC (36%, p<0.05), papillary morphology (50%, p<0.01), histological grade 1/2 (G1/2: 57%, p<0.01) and pTa/is/1 stage (55%, p<0.01) than in those with histological variants (0%), nodular morphology (9%), G3 (16%) and pT2/3/4 (13%), respectively. Patients with ANXA10+ showed better cancer specific survival (CSS) than those with ANXA10- (P<0.05). With IHC, ANXA10+ was detected more in TP53 negative cases (44%, P<0.01) and Uroplakin 3 (UPK3) positive cases (44%, p= 0.089) than in TP53 positive cases (6%) and UPK3 negative cases (28%), respectively. In TCGA dataset of MIBC, higher ANXA10 expression was correlated with papillary morphology, lower grade/stage, luminal papillary subtype and wild type of TP53. ANXA10 expression increases during carcinogenesis, and is observed more frequently in papillary UC with lower grade and stage. However, its expression decreases during cancer progression. Transition of ANXA10 expression in UTUC mimicking BC is clinically useful for decision-making.