Three patients with vitamin D-dependent rickets type II were given massive doses of 1 alpha-hydroxyvitamin D3 for 29 to 36 months and their calcium-phosphate balance was studied during treatment and one month after cessation of treatment. During treatment fasting hypercalciuria was observed in patient 1 and an increased rate of calcium excretion after calcium loading in patients 1 and 2. In these patients, calcium excretion was parallel with the serum 24,25-dihydroxyvitamin D concentration, which may reflect the level of receptor for 1,25-dihydroxyvitamin D. These findings suggested that the responsiveness to 1,25-dihydroxyvitamin D improved during long-term treatment of these two patients with vitamin D-dependent rickets type II.