摘要:Introduction: Malignant mixed tumor (MMT) is an exceeding ly rare cutaneous adnexal carcino ma wit h a sig nificant risk fo r aggressive behavior and a propensit y for metast asis. This tumor occurs in a wide age range and is twice as common in wo man than in men. MMT sho ws a predilect ion fo r the trunk and the extremist's foremo st hands and feet. MMT may be co nfused clinically with many benign and malignant lesio ns. Therefore the histopatholog ical and immunohistochemical examination is required for the correct diagnosis and treatment. Purpose: To present a case o f MMT localized in the left toe with a special attention focused on its histopatholog y and differential diagnosis. Case presentation: A 56-year-old female was admitted with a nodular lesio n on the left to e. A painless mass had been presented for one year with a significant increase in size within the past three month. The patient underwent wide surgical excision. Histopathologically for the lesion was diag nosed it as a malignant mixed tumor. Immuno hist ochemical examinatio n revealed posit ive reactio n for cytokeratin AE1/AE3/PCK26, viment in and S-100. Histochemical react ion for PAS in t he chondromyxoid tu mor stroma was negative. Conclusion: Co rrect histopatholo gical diagnosis and comp lete excisio n wit h wide d isease-free margins before met astasis result in MMT free survival