Pigmented villonodular synovitis (PVNS) is a rare, benign, soft tissue neoplasm affecting the synovium of joints, classified as localized and diffused type. Localized type is more common, arising from synovium of joints, bursae, and tendon sheaths. Diffused type is relatively rare, frequently arising from an extra-articular lesion, and sometimes from an intramuscular or subcutaneous lesion. Although the cause of occurrence is not yet clear, recently it has been known as a benign neoplasm rather than an inflammatory or reactive process. We performed a total excision of the PVNS in a pretibial lesion and achieved a good result. We report on the case with a review of the literature.