Background: Respiratory diseases are common and remain significant causes of emergency in children, especially those less than 5 years old. Objectives: To determine the prevalence, clinical profile, outcome, and common causes of respiratory distress among pediatric patients presenting to the emergency department in King Fahad Hospital, Al-Baha, Saudi Arabia Methods: This was a hospital-based retrospective cohort study carried out at King Fahad Hospital, Al Baha, Saudi Arabia. Children older than 1 month and younger than 14 years who presented to the emergency department with respiratory distress were included in the study. Results: Files of 210 children with respiratory distress were reviewed in this study. The prevalence of respiratory diseases in children was 24%. Hyperactive airway disease was the most common causes of respiratory distress, diagnosed in 48 pts (22.85%), followed by bronchial asthma 45 pts, bronchopneumonia 45 pts (21.41%) each, P< 0.009. No mortality was register in this study. Age group less than 5 years was the most common presenting with respiratory distress, and male were affected more with male to female ratio was 1/1.8, (P<0.007). Tachypnea was the most common sign seen in 87.74% followed by cough in 79.90%. Conclusion: Respiratory diseases were found to be significant cause childhood illnesses and emergency admissions in our hospital. The common respiratory signs were tachypnea, cough, fever, nasal flaring and subcostal retractions. No mortality was documented in this study.