Introduction:

Pigmented villonodular synovitis (PVNS) is a rare clinical entity with higher incidence in women (3:1) and an age of presentation between 20 and 40 years. The hip is a rare site of appearance, being involved in only 15% of all cases.

Case report:

47-year-old male with 10 years of evolution characterized by a limited range of motion, pain associated with an increase in volume and shortening of the pelvic limb. Imaging studies showed destruction of the hip joint and extension to the pelvis. The histopathology report described pigmented villonodular synovitis. His final treatment was left hemipelvectomy.

Discussion:

The presentation of the diffuse pigmented villonodular synovitis of the hip has a lower incidence and a locally aggressive behavior. It is necessary to consider differential diagnoses that include malignant neoplasms. The standard treatment is surgical.