Hypokalemic periodic paralysis (HPP) is a disorder that characterized by attacks of skeletal muscle paralysis depending on the changes in serum potassium levels, and can occur due to primary and secondary causes. One of the secondary causes of HPP is distal renal tubular acidosis (DRTA). DRTA is a disorder that characterized by hypokalemia or hyperkalemia hypercalciuria, metabolic acidosis and alkaline urine. DRTA's clinical symptoms are listed as constipation, nausea, vomiting, kidney and skeletal muscle complications, nephrocalcinosis, urolithiasis and severe hypokalemia crisis. In this case report, we reported a patient who admitted to emergency department with complains of nausea, vomiting, and periodic muscle weakness and was diagnosed with hypokalemic periodic paralysis due to DRTA was presented.