A case of a 21-year-old female with a history of hypokalemia and diabetes mellitus was reported. She had been diagnosed with hypokalemia at 16 years old; a workup then showed hypokalemia, normal creatinine, alkaline urine pH with no proteinuria, combined high anion gap and normal anion gap metabolic acidosis on arterial blood gas, and impaired fasting glucose. She has been on oral potassium supplementation since. Subsequently, she had been diagnosed with diabetes mellitus a few months before the consult for which she was started on metformin. She was admitted due to fever, cough, and dyspnea which were attributed to pneumonia. Upon admission, diagnostics revealed azotemia initially attributed to the infection, combined high anion gap and normal anion gap metabolic acidosis, and ketonuria but random capillary glucose was not significantly elevated ruling out diabetic ketoacidosis. Further diagnostics revealed renal loss of potassium associated with alkaline urine, positive urine anion gap, and nephrocalcinosis which pointed towards type I than type 2 renal tubular acidosis. Additional findings included hypomagnesemia and simple renal cyst consistent with hypokalemic nephropathy. On the other hand, serum C peptide was found to be low, thus diagnostic of type I diabetes mellitus. There is limited available literature regarding the coexistence between type 1 renal tubular acidosis and type 1 diabetes mellitus. It has been suggested to be possibly a part of an autoimmune syndrome. In this case, renal biopsy to identify the histopathology and to determine its potential reversibility, and genetic counseling and testing to identify if there is a common gene affection have been offered, to which the patient did not consent to date.