Background <p>Hypophosphatasia is a rare, inherited metabolic bone disease characterized by impaired activity of tissue-nonspecific alkaline phosphatase leading to pathological mineralization of hard tissue, predisposing to poorly healing fractures. Limited access to enzyme replacement therapy makes the treatment of adults with hypophosphatasia challenging. A few case reports have described a beneficial effect of teriparatide on fracture healing in hypophosphatasia. However, this is the first case of a male patient with pediatric-onset hypophosphatasia, not previously exposed to antiresorptive therapy, treated with teriparatide for bilateral femoral pseudofractures.</p> Case presentation <p>We report a 59-old white man with pediatric-onset hypophosphatasia who developed bilateral femoral pseudofractures that were successfully treated with teriparatide. At the age of 54&#xa0;years, our patient complained about reduced physical activity and pain in both hips and femoral bones. X-ray and later computed tomography scan revealed bilateral femoral pseudofractures. Dual energy X-ray absorptiometry showed normal bone mineral density. Not previously exposed to antiresorptive treatment, our patient was treated with conventional doses of teriparatide (Forsteo^®, 20&#xa0;µg/day, subcutaneous) for 18&#xa0;months. Markers of bone formation and bone resorption increased twofold at 3 months of treatment. At 6 months of treatment with teriparatide normal levels of alkaline phosphatase as well as pyridoxal 5′-phosphate were measured. Early signs of fracture healing were observed at 6 months, and after 18&#xa0;months of teriparatide treatment, the complete healing of both femoral pseudofractures was proven by X-ray and a computed tomography scan.</p> Conclusion <p>Treatment with teriparatide induces beneficial clinical, biochemical, and radiological responses and may provide an efficient treatment option for adults with hypophosphatasia and pseudofractures or poor healing fractures.</p>