Osteogenesis imperfecta (OI) is a rare, inherited disease group with genetic and clinical diversity. There is a defect in the type 1 collagen structure. Increasing bone fragility, osteopenia, and skeletal deformities can be seen. Surgical applications in patients with OI may cause complications such as spinous process injury in vertebrates or bone fracture. Therefore, in the management of pediatric urolithiasis, the decision of surgery should be carefully considered, and safe techniques that shorten the procedure time should be preferred. Percutaneous nephrolithotomy (PCNL) operation is the first choice for large kidney stones in adult patients. Along with the technological advances, more minimally invasive approaches such as mini-PCNL, ultra-mini-PCNL, and micro-PCNL have been developed, and these methods are safely used in pediatric patients due to their low morbidity. Because of its potential risks, urinary stone surgery in OI patients has rarely been reported in the literature. This study presents a 4-year-4- month-old girl with OI and kidney stone, whom we performed ultra-mini-PCNL.