A heart-healthy lifestyle adopted during childhood and sustained throughout life can reduce cardiovascular disease risk in youth with dyslipidemia. In this review, we discuss nutrition recommendations for youth (< 18years-of-age) with dyslipidemia, compare recommendations for youth versus those for adults, review published data regarding nutrition management in the pediatric population, and discuss strategies for successful implementation in a clinical setting. Recent publications highlight the characteristics of genetic and acquired hypertriglyceridemia disorders, dietary adjuncts used for lipid-lowering, and the effectiveness of a multi-disciplinary team approach. Nutrition interventions remain a cornerstone of lipid management and cardiovascular disease risk reduction in youth with dyslipidemia. Nutrition counseling should include age and developmentally appropriate education while also addressing barriers to implementing a heart-healthy lifestyle. A registered dietitian nutritionist plays an important role within a multidisciplinary clinic setting by providing dietary recommendations to address the needs of youth with dyslipidemia.