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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.

<b><i>Purpose of the Study:</i></b> Our aim was to perform an extensive search of the literature on the prevalence of nail psoriasis in the pediatric population and to estimate it on our own database. <b><i>Procedures:</i></b> Searching the most important databases yielded results of 16 papers published in a 40-year period which reported the prevalence of nail psoriasis in children. Furthermore, data gathered between 2004 and 2013 at two centers specialized in nail disorders were analyzed. <b><i>Results: </i></b>The selected papers encompassed a total of 4,853 psoriatic children, of whom 762 (15.7%) had nail involvement. Concerning our data, a total of 68,839 children were seen in pediatric skin consultation in both clinics over a period of 10 years, 0.11% of which had nail involvement. The rate of psoriatic children presenting nail alterations was 19.4% in the Greek and 15.5% in the Italian patient groups. <b><i>Conclusions and Message:</i></b> This is the retrospective study including the largest number of cases of nail psoriasis in the pediatric population. Our data are in line with the incidence estimation of the rest of Europe.