Majocchi's granuloma (MG) is a well-recognized but uncommon infection of dermal and subcutaneous tissues that is caused by mold fungi. Although primarily caused by keratinophilic dermatophytes such as anthropophilic Trichophyton rubrum, species from the Aspergillus and Phoma genera have been occasionally detected as etiologic agents of MG. In both healthy individuals and immunocompromised hosts, MG often presents as nodules, plaques, and papules on areas that are prone to trauma. Although MG generally appears on the upper and lower extremities (forearms, hands, legs, or ankles), it occasionally appears on the scalp and face. The clinical, mycologic, and/or cytologic diagnosis should be confirmed by the demonstration of perifollicular granulomatous inflammation by histologic examination. This review focuses on the clinical presentation, pathogenesis, laboratory diagnostic methods (including the Tzanck smear test), etiologic agents, histopathologic characteristics, and therapeutic approaches to the treatment of MG.