A cultured cell line was derived from a hemangiopericytoma obtained at surgery from a 9 year-old boy with hypophosphatemic rickets, Hypophosphatemia and metabolic bone disease were cured in the patient after tumor removal, Cells from passage 5 were transplanted into 8 week-old athymic female mice. Additional animals received innocula of cells thawed from the stored original tumor tissue, as well as MRC-5 fibroblasts, Serum phosphate levels 3-7 months post-transplantation were lowest in mice which received the cultured cell line (n=5) 6.1+/-0.6 mg/dl (p<0.05 compared to the other groups), thawed tumor cells (n=8) 7.2+/-0.7 mg/dl, MRC fibroblasts (n=4) 8.1+/-1.0 mg/dl, no transplant (n=10) 8.7+/-1.9 mg/dl, Repeat of the experiment with cultured tumor cells from passage 12 no longer altered phosphate levels, A substance produced and released by the tumor in situ and by tumor-derived cultured cells is capable of producing hypophosphatemia. Experimental manipulation of functional tumor-derived cell lines may help elucidate the factor(s) causing hypophosphatemia in oncogenic osteomalacia/rickets.