<p>Solid organ transplantation (SOT) has moved from a rescue option to a planned, disease-modifying therapy for selected inborn errors of metabolism (IEMs). In liver-dominant enzymopathies, transplantation can provide clinically meaningful metabolic capacity and reduce exposure to recurrent catabolic crises; in kidney- or dual-organ phenotypes, it may offer definitive organ replacement while attenuating the downstream consequences of toxic metabolite burden. It is imperative to understand that transplantation does not “cure” many IEMs: extrahepatic enzyme deficiency, established neurologic injury, and chronic systemic complications can persist, and outcomes depend as much on meticulous perioperative metabolic care and long-term multidisciplinary follow-up as on the surgical act itself. This review synthesizes an organ-based, practice-oriented approach to SOT in IEMs, focusing on (i) the therapeutic intent of transplantation (metabolic replacement, end-organ replacement, or risk-reduction), (ii) indications and timing for liver, kidney, combined liver–kidney, heart, lung, and multivisceral transplantation, (iii) sequencing decisions in dual-organ disease, and (iv) peri-transplant metabolic and immunosuppression considerations that are distinctive to IEM. The decision frameworks and succinct conditions where shared decision-making, and context-sensitive practice in resource-variable settings are also of paramount importance.</p>