We present a case of a previously healthy adolescent male who initially presented to his primary care physician with the chief complaint of a “large and white tongue,” who subsequently was diagnosed with end-stage kidney disease (ESKD) and associated uremic stomatitis. This patient required admission to a PICU for acute renal replacement therapy with intermittent hemodialysis, and his hospital course was complicated by generalized tonic-clonic seizures. ESKD is difficult to diagnose in the pediatric population because these patients are often asymptomatic in the early stages given the insidiousness of underlying disorders. Renal disease should be considered in the differential diagnosis of a child with a white tongue not being the result of oral candidiasis.