Renal tubular acidosis is a relatively uncommon clinical syndrome characterized by the inability of the kidney to adequately excrete hydrogen ions, retain adequate bicarbonate, or both. This syndrome can be categorized into 3 separate disorders, each with unique clinical characteristics. Although an uncommon finding, prompt and inexpensive tests can lead to early intervention and subsequently reduce complications from persistent renal dysfunction. The purpose of this article was to bring awareness of the clinical manifestations, diagnosis, and treatments of renal tubular acidosis to critical care nurses.