Renal scintigraphy is a useful tool in diagnosis and management of various nephro-urological conditions. Tc-99m dimercaptosuccinic acid renal scintigraphy (Tc-99m-DMSA), Tc-99m mercaptoacetyltriglycine (Tc-99m-MAG3) or Tc-99m diethylenetriaminepentaacetic acid (Tc-99m-DTPA) dynamic renal scintigraphy, and Radionuclide micturating cystography are the common scans used in children with kidney diseases. These studies are minimally invasive, easily available, and offer both anatomic details and functional information required for thorough evaluation. At the same time, it is essential to have appropriate knowledge to interpret these studies and be aware of their limitations and pitfalls. The advent of Positron emission tomography-computed tomography/magnetic resonance imaging (PET-CT/MRI) has broadened the scope of nuclear medicine. This article focuses on the technique, interpretation, indication and recent practice guidelines of renal scintigraphy in children with kidney diseases.