Objective We aimed to evaluate the clinical profile and radiological findings of children with nutcracker syndrome (NCS) and to assess the association between the parameters. Methods A retrospective analysis of the clinical, laboratory and radiological parameters of children diagnosed with NCS between January, 2011 and October, 2017 was done. Results Of a total of 29 patients [19 girls, 65.5%] with NCS, having a mean (SD) age of 10.8 years, 72.4% had BMI <-2SD. Approximately half of the patients (51.7%) were asymptomatic. Left flank pain was commonest (9/29; 31%) symptom, followed by macroscopic hematuria (4/29; 13.8%). Isolated proteinuria was seen in 9 children. There was no significant difference between the symptomatic and asymptomatic patients in terms of Doppler ultrasonography findings. All patients were followed up conservatively, 5 received enalapril therapy for moderate proteinuria. Conclusion NCS should be considered in children, especially with low BMI, presenting with orthostatic proteinuria and hematuria, with or without left flank pain after ruling out the common causes.