Upper urinary tract fungal infections are rare, under-reported and potentially fatal. Infections often develop in patients with significant co-morbidity and are difficult to identify and treat. They can manifest as local (funguria) or systemic infection (fungaemia). The management is complex and mortality appears unchanged in the past 20 years. Unlike lower urinary tract funguria, which is classified as low risk, upper ureteric infections and fungaemia are classified as high risk.The incidence is increasing and may be associated with changing population demographics, advances in medical diagnostics, and new stent and catheter technologies with longer durations of insertion. We review the current literature and report on six cases.