A 30-year-old female presented to our hospital with trivial injury to her right thigh, following which she had pain over right thigh and unable to weight bear on the involved lower limb. Other associated injuries were ruled out at the time of admission in the emergency room. Physical examination revealed swelling, tenderness, and deformity over the middle-third of her right thigh region without neurovascular deficit. Patient was stabilized initially in our emergency department, and radiograph of right femur showed displaced transverse fracture in the femoral shaft (). The other radiographic findings were diffuse osteosclerosis and narrow medullary canal. The patient gave a history of earlier episodes of fracture for which conservative treatment in form of native splinting had been done. Thus, a diagnosis of pathological fracture was made. On general examination, the patient had short stature and dysmorphic facies with radiological signs of generalized osteosclerosis and narrow medullary canal. A detailed history, clinical, and radiological examination was carried out. She was second child of four siblings. The first child died days after delivery, of unknown cause, and other two younger siblings had similar clinical features (). Parents had a first-degree consanguinity. The antenatal history was not significant. Milestone and intelligence attainted appropriate for age. There was a history of frequent episodes of upper respiratory tract infection, snoring during sleep since childhood. The patient also gave a history of hard of hearing on the right side. The patient went through regular general medical checkups, and there was no significant treatment history. On general physical examination, weight of patient was 63 kg; her standing height was 126 cm; her upper segment and lower segment length were 64 and 62 cm, chest expansion of 3 cm, respectively, with a head circumference of 49 cm. Other clinical features include dysmorphic facies, frontal and parietal bossing, beaked nose, midfacial hypoplasia, short hands and feet with dysplastic nail (), grooved palate (), dental caries, impacted and malposed tooth, and sandal gap deformity of the foot (). Radiograph of the skull was found to be widely open anterior and posterior fontanelle, with obtuse mandibular angle and separated cranial sutures (). Radiograph of the hand showed aplastic terminal phalanges with acroosteolysis. Abovementioned clinical and radiographic findings suggested pyknodysostosis as the most likely diagnosis. The patient was taken up for surgery within a week from injury. An intramedullary interlocking nailing was contemplated initially for fracture fixation. However, for this case, we preferred plate osteosynthesis because of the altered anatomy of the bone with a narrow medullary canal and severe osteosclerosis. Under Spinal anaesthesia, through lateral approach to the middle third of femur, a 12 cm incision was made. After deep dissection, fracture site was identified and exposed. After reducing fractures fragments an “8 holed broad dynamic compression plate with 3 proximal and 3 distal screws in compression mode was used for fracture fixation ().” The difficulty encountered in the intra-operative period include, a difficulty in drilling the cortices and excess blood loss. The wound was closed in layers after adequate hemostasis. The immediate post-operative period was uneventful, and the patient discharged on the 14th post-operative day. The patient was discharged with advice of non-weight bearing walking with the help of walker support for minimum of 2 months and advised to review in the out-patient department for follow up every fortnight. At 10-week post-operative, after patient has been started on partial weight bearing walking, the patient reported with the complaints of pain over surgical site. On radiological examination, patient was found to have no signs of union at the fracture. Patient was immobilized for one month, but even after that, there were no signs of union. Hence, an additional stabilization procedure was done in the form of plating with autogenous cancellous bone grafting. Bone graft harvested from ipsilateral iliac crest was inadequate, and there was heavy bleeding from donor site so that an additional synthetic bone graft substitute was used. In the immediate post-operative period, patient developed deep seated infection which showed organism grown was staphylococcus aureus for which wound debridement was done. Patient was started on Intravenous antibiotics of Linezolid 600 mg twice a day after obtaining culture and sentivity report for period of 2 weeks, followed by oral antibiotics for 4 weeks. Patient was discharged 4 weeks later after complete subsidence of infection. Patient was advised physiotherapy in form of static and dynamic Quadriceps strengthening exercises, followed by hip and knee mobilization exercises and strict non weight bearing for 2 months.. At 3-month post-operative follow-up, there were signs of union clinically and radiologically ( and ). The patient at present in the 6-month post-operative period with fracture united and on full weight bearing carrying out her daily activities without difficulty ().