Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a chronic affliction characterized by numerous liver and kidney cysts. There is a gradual but progressive renal and liver impairment which may require combined liver-kidney transplantation. Compression of the retrohepatic Inferior Vena Cava (IVC) by an enlarged polycystic liver may impede clear visualization on pre-operative imaging and miss an underlying thrombosis or obliteration. This may result in an intra-operative surprise. Management can be challenging requiring modification of conventional surgical approach. We present our experience of a 67-year-old patient who underwent combined liver-kidney deceased donor transplantation for decompensated chronic liver disease with chronic kidney disease due to ADPKD. She was diagnosed with ADPKD for 16 year, with progressive deterioration in kidney function over the last 6 year and liver decompensation following knee replacement surgery requiring regular renal replacement therapy. We report this case to highlight the peri-operative challenges and their management along with a review of published literature on this uncommon occurrence.
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