A combination of osteoporotic vertebral fractures and congenital spinal deformity is theoretically possible, but there have been no reports on this combination in the literature. We describe a rare case of an osteoporotic vertebral fracture adjacent to the nonsegmented hemivertebra. A 60-year-old postmenopausal woman who did not recall any specific trauma presented with severe back pain. She had markedly decreased bone mineral density and significant lumbar kyphoscoliosis with a nonsegmented hemivertebra between L1 and L2 on radiographs of the lumbar spine. Magnetic resonance imaging (MRI) revealed a vertebral fracture adjacent to the nonsegmented hemivertebra. Laboratory studies showed increased serum bone-specific alkaline phosphatase (BAP) and urinary type I collagen crosslinked N-telopeptide (NTx). A thoracolumbar brace was applied for 3 months. Daily administration of alendronate normalized her serum BAP and urinary NTx levels. MRI scans of the lumbar spine after 6 months also confirmed normalized signal intensities of the fractured vertebra adjacent to the nonsegmented hemivertebra. The vertebral fracture seemed to be induced by spinal malalignment, increased stress on the adjacent level of the fused segment, and its fragility due to osteoporosis.