Nevus spilus (NS), also known as speckled lentiginous nevus, is characterized by background café au lait-like lentiginous melanocytic hyperplasia speckled with small, 1- to 3-mm, darker foci. Nevus spilus occurs in 1.3% to 2.3% of the adult population worldwide. Reports of melanoma arising within hypertrichotic NS suggest that hypertrichosis may be a marker for the development of melanoma. We present a case of a hypertrichotic NS without melanoma and also provide a review of previously reported cases of hypertrichosis in NS. We believe that NS has a lower risk for malignant degeneration than congenital melanocytic nevi (CMN) of the same size, and it is unlikely that hypertrichosis is a marker for melanoma in NS.