Onychomatricoma is a rare benign fibroepithelial filamentous tumor originating from the nail matrix. It typically presents with the clinical tetrad of xanthonychia, pachyonychia, proximal splinter hemorrhages and increased transverse overcurvature of the nail plate. The giant variant can easily confuse the clinician due to its extensive nail dystrophy that can mask the characteristic features of this tumor. Benign (fibrokeratoma, ungual fibroma, onycholytic matricoma) and malignant entities (Bowen's disease, squamous cell carcinoma, onycholytic carcinoma) are mimics of the disease. Nail surgery can facilitate the diagnosis, which should always be confirmed by histology, as rare variants do exist.