We report the case of a young female patient who received a total hip replacement due to pain in her left hip joint, misdiagnosed as degenerative arthritis. A clear cell chondrosarcoma (CCCS) in the femoral head had initially not been detected. Seven years later, a periprosthetic recurrence of CCCS close to the former femoral osteotomy occurred. Although a sample of the periprosthetic tumour had been taken for histologic analysis, the recurrence of CCCS remained misdiagnosed for almost 2 years until this rare histopathologic morphology was examined in a specialised multidisciplinary tumour centre. Finally, revision surgery with wide tumour resection margins had to be performed requiring the removal of the total hip replacement and its reconstruction using a modular megaimplant (proximal femoral replacement). Relevant facts of the CCCS as a rare entity regarding histology, treatment and differential diagnoses are discussed.