Onychomatricoma is a rare tumor originating from the nail matrix, and, in rare conditions, from the ventral aspect of the proximal nailfold. Here we report a rare case of a 51-year-old man presenting with melanonychia mainly involving the distal nail plate. Histopathologic examination showed typical findings of onychomatricoma mainly involving the nail bed, while the nail matrix was largely uninvolved. We also identified fungal infection in a focal area of the distal nail plate. Our findings indicate that onychomatricoma can develop in the surrounding epithelial tissue of the nail unit, including the nail bed, and suggest that fungal infection may represent a secondary phenomenon of onychomatricoma.