Within a period of 3 years, 103 patients with primary type I distal renal tubular acidosis were reported from two hospitals in the northeast of Thailand, with cases peaking in the summer. The patients' age ranged from 18 to 76 with a mean and mode of 39 and 47 years, respectively. The female to male ratio was 3.3:1. All patients presented with generalized muscle weakness and bone pain and nocturia were frequent manifestations. Hypokalaemia and hyperchloraemic metabolic acidosis were constant findings and were more pronounced in patients with roentgenographic evidence of nephrocalcinosis and osteomalacia. Urinary citrate was extremely low in most of the patients. Nephrocalcinosis and/or renal stones were present in 27.2% of the patients and osteomalacia was found in 23.3%. All patients were Lao-Thai. This is the first report of a large group of patients with distal renal tubular acidosis, endemic within a particular geographical region.