Onychomatricoma (OM) is a rare benign tumour of the nail matrix characterized by specific clinical and histologic features. The main clinical signs are thickening of the nail plate, xanthonychia, overcurvature of the nail plate, and multiple splinter haemorrhages. The diagnosis is based on clinical, radiological and histopathological findings. Histologically, the tumour is characterized by filiform epithelial projections. The objective of this study is to present the first reported Tunisian case of OM, focusing on the contribution of magnetic resonance imaging to the diagnosis of OM. A review on the subject is also presented.