Pigmented villonodular synovitis (PVNS) is a proliferative lesion of the synovium, mainly occurring in the knee joint. PVNS in the hip joint is less common than in the knee joint, and can cause joint effusion and swelling, damage joint cartilage, and affect joint function. This article provides a detailed report on a patient who was initially diagnosed with femoral head necrosis. During total hip arthroplasty, villous synovial hyperplasia was found in the hip joint, and postoperative pathology confirmed pigmented villonodular synovitis. This case provides relevant experience for the clinical treatment of this disease, especially in terms of preoperative diagnosis. For cases of rapidly progressing femoral head necrosis, PVNS should be considered as a differential diagnosis, and MRI or joint biopsy should be performed to assist in the diagnosis.