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Introduction: Perivascular epithelioid cell (PEC) is a cell type constantly present in a group of tumours including angiomyolipoma (AML), clear-cell «sugar» tumour (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis (LAM), and clear-cell tumours of other anatomical sites. It has morphologic distinctive features: epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus and an inconspicuous nucleolus. Immunohistochemically, PEC expresses myogenic and melanocytic markers. Eleven cases of primary bone PEComa presentation have been described since 2002., Objective: To report a case of primary bone perivascular epithelioid cell tumour., Case Report: 24 year-old male presented with pain. X-ray revealed an osteolytic lesion at right proximal tibia with soft tissue extension. Evaluation of slides identified a bony perivascular epithelioid cell tumour without immunohistochemical study confirmation., Results: Patient was treated by surgical excision and adjuvant chemotherapy (epirubicin/cysplatin). After two years of follow-up the patient remains disease free., Conclusions: This is the first-case report in Latin America. Immunohistochemical stains were negative and we believe it may be due to non-described ethnic variations., (Copyright © 2018 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.)