Background:DFSP is a rare soft tissue tumor with indolent growth and low probabilityrnof distant metastasis. More than 90% of cases are positive for CD34 and are characterizedrnby translocation of chromosome 17 and 22 (t (17; 22)) resulting in the expression ofrnfusion gene-COL1A1-PDGFRb. A TKI, imatinib targets this fusion gene and showedrnefficacy in treating both locally advanced and metastatic DFSP.rnObjective:The aim of this study is to describe the clinical characteristics of patients andrnto evaluate response to imatinib of patients with DFSP treated in Tikur Anbessa Hospital.rnMethods: We assessed retrospectively 26 patients with DFSP treated with imatinib fromrn2004-2020 at hematology unit of Tikur Anbessa Hospital, in Addis Ababa. Clinical datarnwas extracted from medical records and patients and analyzed with SPPS version 26.rnResponse to imatinib was evaluated using Response Evaluation Criteria in Solid Tumorsrn(RECIST).rnResults:18 of the patients were males and 8 were females with a median age of 35 yearsrn(range: 22-66). Trunk is the commonest location of primary tumor constituting 61.5%,rnfollowed by head and neck. Twelve patients have been treated with imatinib for locallyrnrecurrent disease, which was difficult to resect surgically, and 6 (50%) showed partialrnresponse, 3 (25%) complete response, 1 (8.33%) patient had stable disease and 2rn(16.67%) had disease progression. On the other hand, thirteen patients underwent surgeryrnfor locally recurrent disease followed by imatinib therapy for residual disease. Six (6) ofrnthese patients had complete response, 3 patients had partial response and 4 patients hadrnprogressive disease while on imatinib. One patient had recurrent disease with liverrnmetastasis and he underwent surgery for local recurrence and imatinib was given for liverrnmetastasis which disappeared after 3 months. The overall clinical response rate wasrn73.1%. The major adverse effects of imatinib were: GI upset in 38.5%, skinrndepigmentation in 34.4%, anemia in 23%, leukopenia in 23% and edema in 19.2%, whichrnwere all transient and self-limiting.rnConclusion: DFSP is highly recurrent tumor after surgical resection. We found out thatrnImatinib is well tolerated and an effective treatment option for locally recurrent andrnadvanced disease that is difficult for complete surgical resection, with over all clinicalrnresponse of 73.1%.