Onychomatricoma is a primary benign neoplasm of the nail matrix. Currently, a limited number of cases have been reported, so it is still considered a rare neoplasia. However, it is debatable if this condition is underdiagnosed and underreported. Onychomycosis is an important differential diagnosis of onychomatricoma, and sometimes, both these conditions may even coexist in the same nail. As the tumor grows, tissue microenvironment is more vulnerable to dermatophytes. Probably, the altered keratin appears to be susceptible to fungal invasion. Careful clinical assessment and dermoscopic evaluation help nailing the diagnosis. Usually, total nail avulsion is the preferred therapeutic approach when they coexist. Herein, we present a case of a middle-aged woman with onychomycosis and onychomatricoma affecting a single fingernail. The proposed therapy was oral terbinafine for 6 months followed by a conservative surgery. There were dramatic changes in dermoscopic features after fungal treatment, which, to our knowledge, have not been previously reported.
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