Purpose of reviewIn a child with evidence of acute kidney injury (AKI) following renal transplantation, it is important to quickly and accurately diagnose the cause to enable timely initiation of therapeutic interventions. The following article will discuss the differential diagnosis of acute graft dysfunction in paediatric kidney transplant recipients. This review will systematically guide the clinician through the common and less common causes and provide updates on current treatments.Recent findingsIn patients with signs of graft dysfunction, rejection is an important cause to consider. Diagnosis of rejection relies on biopsy findings, an invasive and costly technique. Over the past 5 years, there has been a focus on noninvasive methods of diagnosing rejection, including serum and urinary biomarkers.SummaryThis review discusses the differential diagnosis of acute graft dysfunction following transplant, with a focus on acute rejection, urinary tract infections and common viral causes, prerenal and postrenal causes, nephrotoxic medications, specifically calcineurin inhibitor toxicity, thrombotic microangiopathy and recurrence of the underlying disease. Each condition is discussed in detail, with a focus on clinical clues to the cause, incidence in the paediatric population, workup and treatment.