Background: Hirschsprung’s disease (HD) is the most common surgical condition in pediatrics posing challenges to pediatricians and pediatric surgeons mainly practicing in resource-limited countries. Clinical features of the disease are variable and the risk factors in our setting are not well studied. Objectives: This study aimed at describing the clinical characteristics and determining the risk factors for Hirschsprung’s disease among Hirschsprung’s disease patients at Muhimbili National Hospital. Method: A case-control study was carried out whereby patients with histologically confirmed Hirschsprung’s disease and their caretakers admitted to the ward or attending regular clinics were selected, and verbally interviewed using a structured questionnaire and their findings were compared with corresponding controls without HD. Results: The study enrolled 225 cases and 476 controls. The majority of participants were male accounting for 156(69.4%) and 286(63.4%) for both cases and controls respectively. The mean age of the participants was 58 months, mainly coming from the coastal region where the hospital (MNH) is located. The most common age at first clinical presentation was neonatal age but the most age at confirmatory diagnosis was 25-60 months. Chronic constipation was the most common clinical presentation accounting for 223(99.1%) of cases followed by abdominal distension 207(92.0%) and soiling. Failure to pass meconium within 24 hours was common in cases (37.6%) compared to control (8.6%). %). Prematurity, Down’s syndrome, history of HD, and congenital anomalies in the family were found to be associated risk factors for Hirschsprung’s disease. Conclusion: The most common clinical presentations were found to be chronic constipation, abdominal distension, failure to pass meconium within 24-48 hours of life, and male predominance. Prematurity, Down’s syndrome, history of HD, and congenital anomalies in the family were more likely to be associated with Hirschsprung’