Case: A 40-year-old Colombian woman presented with a 7-year history of progressive lower-limb pain. Sclerosis of the diaphyseal tibia and femur was observed in her latest x-ray images. A narrowing of the medullary canal is observed in Camurati-Engelmann disease (CED), a rare and progressive diaphyseal dysplasia that was confirmed in this patient by genetic testing. Medical treatment was unsuccessful; thus, surgical treatment consisted of decompression by drilling of the medullary canal was performed, achieving successful pain release. Conclusion: Surgical treatment should be considered for patients with CED when the medical treatment is unsuccessful because doing so reduces bone overgrowth, leading to pain relief.