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To investigate the incidence and mortality trends of upper tract urothelial cancers (UTUC) in Victoria over the last decade.Age-adjusted incidence and mortality rates were calculated for UTUC. These were identified using data from the Victorian Cancer Registry from 2001 until 2011 based on histological diagnoses. Age at diagnosis, sex and demographical location were compared.The age-standardised incidence of UTUC remained stable from 2001 to 2011. There were 278 deaths from UTUC over this period with an overall 5-year survival rate of 32%. There was no significant difference in survival between 2001–06 and 2007–11 (30% vs 36%, respectively). Lower age at diagnosis was associated with a significant improvement in survival (P = 0.01). Sex and geographical location appeared to have no effect on survival.The 5-year survival rates for UTUC in Victoria are poor, particularly in comparison to worldwide data. In contrast to worldwide trends, the incidence of UTUC appears to be stable. No significant improvement in 5-year survival rates over the short study period was identified. These findings highlight the difficulties in managing this rare yet deadly malignancy.