Wilson disease is a rare autosomal recessive genetic disorder of copper metabolism that leads to copper accumulation and subsequent organ dysfunction. While classically considered a condition that primarily affects the liver and nervous system, Wilson disease and its treatments can also result in a wide range of kidney complications as well. We present the case of a 31-year-old female with a longstanding (>10 year) history of Wilson disease who developed acute-onset nephrotic syndrome including heavy proteinuria, hypoalbuminemia, and edema after being transitioned from zinc to D-penicillamine for copper chelation therapy. Following simple cessation of D-penicillamine (and without any immunosuppressive therapies including corticosteroids), the nephrotic syndrome showed remarkable improvement including complete remission within several months. This review comprehensively summarizes the kidney complications associated with Wilson disease and its treatments.