Renal candidiasis is an increasingly common condition affecting predominantly premature infants receiving neonatal intensive care or term infants with urogenital tract anomalies. Multiple risk factors are usually present. Although rare, some infants develop an obstructive uropathy due to fungal balls, and this requires prompt detection and intervention to preserve kidney function. The management of obstructive renal bezoars is challenging and not well summarised in the past. This is mainly due to scarce literature confined to case reports or case series only. This review clarifies various definitions used in relation to renal candidiasis and identifies infants particularly at risk of obstruction. Clinical presentation, diagnosis and the role of imaging are discussed. A summary of the recent literature is provided to outline the range of existing treatment options available with published drug dosages and mode of delivery used. No single approach is successful in all cases and clinicians need to be aware of the different options available: apart from adequate urinary drainage and use of systemic +/- local antifungal agents, additional treatment with fibrinolytic agents and/or endoscopic or open surgical removal may be required. A new simplified algorithm for use in management is proposed. We hope this review will help clinicians in their management of patients presenting with this complex and challenging diagnosis.