Purpose of Review <p>Dyslipidemia can present as early as infancy however the prevalence and long-term outcomes are unclear. There is an unmet need for guidance in the evaluation and treatment approach in these patients. This review summarizes the pathophysiology of dyslipidemia in infants and toddlers and highlights potential treatment options.</p> Recent Findings <p>Secondary factors unique to this population including prematurity and reliance on intravenous nutrition play a role in the pathophysiology of dyslipidemia, though primary genetic causes are also recognized. Severe hypertriglyceridemia poses a risk of acute pancreatitis in an already vulnerable population. Persistent dyslipidemia is a concern for future premature cardiovascular disease.</p> Summary <p>Management of dyslipidemia is dependent on its etiology and severity. Primary and secondary causes should be considered and addressed. Although a variety of therapeutic agents are available in older children and adults, no approved therapies exist at this age, though off-label use of medications may be considered.</p>