Nail matrix nevus is a type of benign melanocytic proliferation that presents as longitudinal melanonychia (LM) [1]. Nail matrix nevus may be congenital or acquired and is localized to a fingernail or toenail [2, 3]. Histopathologically, there is a melanocytic proliferation organized into nests. Nail matrix nevus variants include junctional, compound, or intradermal.