Objective　To explore the pathogenesis, clinical characteristics and treatment of pseudohypoparathyroidism (PHP) Ia. Method　The clinical and genetic data of PHP Ia in a child and her family members were retrospectively analyzed. Results　The child had congenital hypothyroidism as first symptom, and then gradually had Albright hereditary osteodystrophy (AHO), low calcium, high phosphorus and resistance to parathyroid hormone. After treatment with calcitriol and calcium, blood calcium and phosphorus were close to normal, but there was no significant change in AHO malformation. Conclusion　PHP combined with congenital hypothyroidism is rare.