Background: Histiocytic sarcoma (HS) is a rare hematologic malignancy with morphologic and immunophenotypic evidence of histiocytic differentiation. This tumor follows an aggressive clinical course.

Case Presentation: We report the case of a 14 year-old white girl who presented with a nodular lesion in the thigh, involving the skin and soft tissue. The histologic diagnosis retained was a HS.

Conclusion: HS is a rare neoplasm that may cause a diagnostic pitfall. Unfortunately, incomplete clinical data and histopathologic disparities in addition to the overall rarity of the neoplasms induced difficulties of management and of full appreciation of their clinical behavior.

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