Homocystinuria (HCU) is a rare genetic disorder characterized by a deficiency in the enzyme cystathionine β-synthase (CBS), resulting in impaired metabolism of the amino acid methionine and elevated levels of homocysteine. With an estimated incidence of 1 in 100,000 live births globally, HCU poses significant challenges in clinical management due to its multisystemic manifestations and potential for severe complications if left untreated. The disorder affects individuals of all ethnic backgrounds, presenting a wide spectrum of clinical symptoms, from mild to life-threatening complications. Vascular complications, skeletal abnormalities, ocular manifestations, and neurological deficits underscore the complexity of managing HCU. Cardiovascular complications, including premature atherosclerosis and thromboembolic events, are major contributors to morbidity and mortality. Skeletal manifestations such as osteoporosis and ocular complications like ectopia lentis are common features, while neurological manifestations encompass intellectual disability and psychiatric disturbances. Early diagnosis through biochemical and genetic testing is crucial for prompt intervention to prevent or minimize complications. Current management strategies focus on a multifaceted approach, including dietary restriction of methionine, supplementation with vitamins and betaine, and close monitoring of metabolic parameters. Emerging dietary strategies offer promise for improving outcomes in HCU management. These include exploring the optimal balance between methionine restriction and amino acid supplementation, personalized dietary plans tailored to individual metabolic profiles, targeted nutrient interventions, integration of bioactive compounds and functional foods, leveraging technology for monitoring and support, and fostering collaborative research initiatives. By advancing our understanding of HCU pathophysiology and developing novel treatment modalities, we can strive to improve the quality of life and long-term prognosis for affected individuals.