Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disorder affecting synovial membranes, bursae, tendons, skin and bone. Materials The article presents a rare clinical case treated for PVNS of the subacromial space. The patient complained of pain in the shoulder joint at the largest possible points, and a deformity developed 7 months prior to the visit could be visualized at the anterior aspect of the shoulder joint. The patient denied any history of trauma. Arthroscopy of the shoulder joint was performed to rule out the intraarticular nature of the lesion with an exploratory puncture performed to establish the diagnosis with instrumental methods. Delto-pectoral approach was used to remove the neoplasm that was histologically examined to confirm the diagnosis. The upper limb was postoperatively immobilized with a Gilchrist-bandage for 2 weeks. The sutures were removed 2 weeks after the operation. Result The patient underwent a course of rehabilitation to restore the function of the upper limb. The upper limb function was preoperatively and postoperatively evaluated with the ASES questionnaire. The patient showed the shoulder function completely regained at two years with ASES scored 100. MRI scan of the shoulder joint showed no signs of recurrence. Extra-articular location of PVNS is very rare and is described in few case reports. Open procedure or arthroscopic removal of the affected synovial membrane are used to treat the condition. Conclusion Removal of the affected synovial membrane allowed us to obtain a good clinical result © Ryazantsev M.S., Magnitskaya N.E., Ilʼin D.O., Logvinov A.N., Kadantsev P.M., Frolov A.V., Olchev A.A., Korolev A.V., 2022