<h4>Introduction</h4>Pathogenic variants in myosin heavy chain 9 (<i>MYH9),</i> encoding the heavy chain of nonmuscle myosin IIA (NMMIIA), cause autosomal-dominant <i>MYH9</i>-related disease that may include proteinuric kidney disease, macrothrombocytopenia, cataract, sensorineural deafness, and elevated liver enzymes.<h4>Methods</h4>Whole exome sequencing and segregation analysis were performed in a patient with end-stage renal disease. The gene discussed is MYH9; the disease is stage 5 chronic kidney disease.