Terminology regarding classification of benign lesions with prominent eccrine differentiation can be confusing as these lesions can have overlapping clinical and histologic characteristics. In this report, we examine a case and review of the literature to suggest that these entities may be better classified as a spectrum of benign lesions with overlapping features rather than distinct entities. We describe a case of an acantholytic dyskeratotic epidermal nevus with eccrine differentiation on the back of a 2-year-old patient. We then discuss the classic clinical and histologic presentations of eccrine nevi and epidermal nevi with eccrine differentiation as they relate to each other and to our case.