Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia’s Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.