Hirschsprung disease (HD) is a functional bowel obstruction due to the absence of ganglion cells in the distal colorectum commonly or involving more proximal bowel to varying extents less often. It must be differentiated from other causes of constipation in neonates, infants, and children. A clinical diagnosis is followed by screening investigations (imaging , manometry) and confirmed by histopathology prior to a tailored surgical management (bowel stoma/pull through). With early diagnosis and optimal surgical care, a satisfactory functional outcome is achieved in the majority.