Background and Objectives: Haematuria or blood in urine is a scary but common medical problem in children. While microscopic hematuria has been well studied and reported, there has been paucity of data regarding the occurrence and profile of macroscopic hematuria in children. This study was conducted to study the prevalence, demographic, clinical, laboratory and etiological profile of macroscopic hematuria in children. Methodology: The present study was conducted in Pediatrics deptt of our tertiary care level teaching hospital over 2 years from January 2020 to December 2021 including children between 1-16 years presenting with complaint of reddish, cola coloured or reddish black urine. Results: 71 children with true macroscopic hematuria were enrolled out of which 41 were males and 30 were females with male: female ratio of 1.4:1. Mean age of the study participants was 6.53 ± 3.1 years and mean weight of the children was 16.7 ± 3.9 kg. Prevalence of macroscopic hematuria was found to be nearly 4.9 per 1000 children visits. Glomerular hematuria was found in 46 or 64.8% of the children while the rest 25 or 35.2% had hematuria of non-glomerular origin. Overall, the most common aetiology of macroscopic hematuria was post-infectious glomerulonephritis (26.8%) followed by HSP (8.5%), HUS (7.1%), renal stones (7.1%) hypercalciuria (5.6%) and bleeding disorders (5.6%). At 1 year follow-up, persistent microscopic hematuria was seen in 5 (7.1%) and 7 (9.9%) children still required antihypertensive therapy. Conclusion: Gross hematuria is a relatively uncommon condition but its occurrence is most frequently indicator of a serious underlying pathology as glomerular hematuria is the predominant aetiology in children which mandates careful monitoring and long-term follow-up.