Clinical features of Bowen's disease of the nail apparatus are diverse and often mislead clinicians to wrong a diagnosis. We report a case of 45-year old man with a yellowish thickened nail plate accompanied by subungual hyperkeratosis of the right index finger, mimicking onychomycosis. There were two narrow longitudinal pigmented streaks on the nail surface and keratotic papillary projections underneath the distal free edge. After confirming the diagnosis of Bowen's disease by biopsy from keratotic papillary lesion, we entirely removed the nail apparatus followed by full-thickness skin-grafting. HPV DNA was detected in the specimen by PCR using the GP5/GP6 consensus primers. Analysis of the PCR product using direct DNA sequencing revealed that the sequence corresponded to the L1 gene of HPV type 16. No fungal elements were detected by Periodic acid-Schiff (PAS) staining. We reviewed the literature on Bowen's disease of the nail apparatus published since 1987 and summarized 14 patients that clinically mimieked onychomycosis out of 161 reported cases. Most patients exhibited longitudinal streaks or keratotic nodules in addition to clinical features resembling onychomycosis. Subungual Bowen's disease is one of the most important diseases which should be distinguished from onychomycosis.