The objective was to demonstrate a clinical case of an adult patient with Olier’s disease (enchondromatosis), severe hand deformity, and its treatment in orthopedic department. Patient K., 71 years old, was treated with severe deformation of the left hand due to Ollier’s disease in Ya. L. Tsiv’yan Novosibirsk Research Institute of Traumatology and Orthopedics in 2017. The patient underwent a one-stage multi-stage surgical intervention with removal of the endochondromas of the 1st, 2nd, 4th and 5th fingers, bone and tendon grafting of the defects.In 5 years after surgery, patient notes a high degree of satisfaction with the treatment. At the control visit, the preservation of the intraoperatively achieved result was established without signs of recurrence of the process or signs of malignancy. The achieved result led to the improvement of motor skills, restored a number of social self-service functions.Due to the high risks of recurrence and malignancy of the process, patients with Olier’s disease require close monitoring by clinicians. Surgical treatment of benign forms of the disease involves the implementation of an organ-preserving intervention with the reconstruction of bone and tendon ligament defects. The main goals of the intervention in this case were a removing of the pathological substrate and the improvement of hand function.