Background: Malignant proliferating trichilemmal cyst is a rare tumour usually located on the scalp of elderly women. About 40 cases of malignant proliferating trichilemmal tumour have been documented.
Case report: We report a case of a malignant proliferating trichilemmal cyst of the scalp in a 57-year-old woman. On the vertex she had a voluminous vegetated and multinodular tumour measuring 7 x 5 cm with spontaneous and abundant bleeding, and another lesion of the scalp corresponding to a trichilemmal cyst. Based on histopathological findings, the case was diagnosed as malignant proliferating trichilemmal cyst.
Conclusion: Diagnostic, clinicoprognostic and histological features of this tumour are discussed. Treatment is not yet standardized given its rarity.