Neurofibromas are peripheral nerve sheath tumors. They are composed of a mixture of cell types including Schwann cells, fibroblasts, and immune cells [1]. Neurofibromas are most commonly found in or near the skin, often extending along a nerve or involving multiple nerve fascicles [2]. Rarely, they may affect the digits and nails. Subungual neurofibromas may arise in association with neurofibromatosis I (NF1) or as solitary lesions [3] and are exceedingly rare with approximately 15 reported cases [4–7]. There are two main types of neurofibromas: cutaneous (cNF) and plexiform (pNF) [2]. cNF are benign, are found exclusively in the dermis, and are typically present during puberty. In contrast, pNF carries a risk for sarcomatous transformation and can be found deeper below the dermis. They are congenital and progress during childhood and adolescence. Subungual neurofibromas typically present as a myxoid histopathologic variant of cNF [8–10].