Children with Cystic Fibrosis (CF) face a unique challenge when it comes to obesity. CF is a genetic condition affecting the lungs and digestive system, causing thick mucus buildup in the airways and impairing nutrient absorption. Paradoxically, despite malabsorption issues, some children with CF can be prone to obesity due to factors like improved treatments, dietary adjustments, and better disease management. The complexities arise as CF requires a high-calorie diet to combat malnutrition, which, if not monitored meticulously, can lead to excessive weight gain. This dilemma creates a delicate balance between meeting nutritional needs and preventing obesity-related complications. Treatment strategies involve specialized diets rich in healthy fats and calories, along with pancreatic enzyme supplements to aid digestion. However, managing weight in CF children necessitates close monitoring by healthcare professionals to prevent excessive weight gain while ensuring proper nutrient intake. Obesity in CF children can exacerbate respiratory symptoms, strain the already compromised lungs, and heighten the risk of other health issues. Therefore, a multidisciplinary approach encompassing nutritional counseling, exercise regimens, and regular health assessments is crucial to maintain optimal weight and overall well-being in these individuals. Balancing nutritional requirements while averting obesity remains a challenging but essential aspect of managing cystic fibrosis in children.