Plexiform fibrohistiocytic tumor is a rare soft tissue neoplasm with no long-term series reported in literature. A detailed review revealed sixteen patients treated at our institution over a twenty-year (1983-2003) period. In eight patients the location of tumor was in upper extremity and this will be the focus of our report. The most common upper extremity site was wrist, followed by fingertip and upper arm. The median age at presentation was 10.5 years (range: 2-15 years) and had a strong female predilection (F: M, 7:1). The most common presentation was a small painless mass. Grossly the tumor was firm and and histologically characterized by a plexiform proliferation of histiocyte-like cells. All patients underwent wide local excision. Follow-up data is available from 6 patients for an average period of 5.9 years. Of the lesions in upper extremity, the local recurrence was noted in 4 (66%) patients, regional recurrence in 1 (17%) and systemic recurrence in 3 (50%). In conclusion, this is a rare neoplasm characterized by low-grade behavior, tendency for local recurrence as well as regional and systemic metastasis.