Abernethy malformation is a rare disorder defined by congenital portosystemic shunt. Advances in clinical imaging have led to increased identification of this anomaly, which has proven to be more common and more clinically diverse than previously assumed. Late presentations are not uncommon. We present a 35-year-old patient with type Ia Abernethy malformation and biopsy-confirmed mesangiocapillary glomerulonephritis who was referred for deceased donor kidney transplantation. After the diagnosis was confirmed, the patient remained stable and asymptomatic on a supervised low-protein, high-carbohydrate diet. The patient received the kidney transplant from a brain-dead donor with standard characteristics. The procedure was uneventful; no vascular or vesical abnormalities could be identified at the surgical site. Recovery was uneventful with excellent graft function. Unique issues with immunosuppression were identified. Pharmacologic adjustments accounting for congenital complete portosystemic shunting affecting liver first pass effect as well as multiple drug interactions were necessary and sufficient. Abernethy malformation may follow indolent course into adulthood and may be an unrelated finding in a patient with chronic kidney disease. Kidney transplantation proved to be feasible and safe in this young male with apparently efficient compensatory mechanisms.
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