The wide spectrum of distal renal tubular acidosis (RTA) has been reported. Children with distal RTA can present with failure to thrive, gastrointestinal symptoms, nephrocalcinosis, and renal failure, etc. So far, secondary erythrocytosis in pediatric patients with distal RTA has not been reported. Here we report a case of distal RTA with failure to thrive and nephrocalcinosis accompanied by secondary erythrocytosis in a 3-year-old boy. He has been followed up for 2 years and is under treatment with NaHCO3 and potassium citrate. The treatment contributes to the improvement of metabolic acidosis and his growth. There is normal renal function and constant erythrocytosis during the follow-up period. Further studies are needed to clarify the relationship between erythrocytosis and distal RTA.