A B S T R A C T Plasma calcitonin (CT) was measured in the basal state and/or during provocative tests of hormone secretion in 11 children with congenital nongoitrous cretinism (CNC), in 1 girl with a lingual thyroid, and in 11 normal children. Basal and stimulated CT concentrations were significantly lower in the patients with CNC than in the normal subjects. Mean basal CT (± SE) was 41±4 pg/ml in the normal children, 24±3 pg/ml in the children with CNC, and 20±2 pg/ml in the patient with the lingual thyroid. The mean incremental CT responses to calcium infusion were 7.0±2 pg/ml in the children with CNC, 6.0 pg/ml in the patient with the lingual thyroid, and 146±47 pg/ml in the normal children. The children with CNC also demonstrated a significant delay in the return of the total serum calcium to basal level after the calcium infusion. The mean incremental CT response after infusion of pentagastrin was 7.6+2 pg/ml in the children with CNC, 10.0 pg/ml in the child with the lingual thyroid, and 34.4±11 pg/ml in the normal children. These data indicate that CT deficiency is present in children with CNC and suggest that the deficiency is a consequence of the defective embryologic development of the thyroid gland.