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Diagnosing tyrosinemia type 1 can be a challenge as it is a heterogeneous disorder with a wide variety of clinical manifestations and complications. We herein report a rare case of a three-day-old male neonate with HT-1 compounded with HH.Ondansetron (ODSN) is a commonly used and well-tolerated anti-emetic used during pregnancy. Upon review, three cases were identified in which liver function tests (LFTs) were elevated after ODSN administration without concurrent chemotherapy. In this report, we present a case of a 28-year-old pregnan