Melorheostosis is a rare disease that usually burdens the patient with painful disability or soft tissue compromise. The treatment is usually symptomatic and conservative. Patients with severe and complicated forms of the disease may require surgery. Involvement of the distal part of a limb usually carries more morbidity, such as tumefaction pain, cosmetic and psychosocial or functional problems that render conservative treatment unsatisfactory to patients. In our series, surgical debulking or decompression of the mass effect provided prompt symptom relief.