Cutaneous leiomyosarcoma is a rare tumour, accounting for 2,3% to 6,5%
of all soft tissue sarcomas. The case of a young patient with a cutan
eous leiomyosarcoma on the right gluteal region is here presented. The
lesion misdiagnosed as a keloid after primary excision of histiocytom
a had a two-year history, had been excised twice. The tumour was widel
y excised, and the wound closed primarily. No recurrence or distant me
tastasis are reported 26 months after the initial presentation. Clinic
al, histological, immunohistochemical features and surgical strategies
of the reported case are discussed and compared with literature.