Argininemia is a rare autosomal recessive metabolic disorder characterized by a deficiency of arginase, a vital enzyme in the urea cycle. This metabolic defect results in the accumulation of arginine and its metabolites, leading to hyperammonemia and associated neurological symptoms. We present a case detailing the perioperative management of an 11-year-old male child diagnosed with argininemia undergoing circumcision. The perioperative management of patients with argininemia presents unique challenges due to the risk of hyperammonemia and neurological decompensation triggered by physiological stress, fasting, and the catabolic state associated with surgery. This case report highlights the importance of individualized anesthetic strategies for patients with rare metabolic disorders like argininemia. A multidisciplinary approach involving collaboration among anesthesiologists, endocrinologists, dietitians, and surgeons is essential to ensuring a safe perioperative experience for these patients. Further research is essential to refine perioperative protocols and optimal anesthetic interventions for individuals with argininemia undergoing surgical procedures.