A 15-year-old man presented with a solitary hairy patch on his right cheek that was first noticed 7 years ago. One month prior, he had been diagnosed with neurofibromatosis type 1. On examination, multiple cafe-au-lait macules, neurofibromas and axillary freckling were observed. Histopathologic examination revealed a number of pigmented ovoid to elongated epithelioid cells in the deep dermis. Based on the clinical and histopathological features, the diagnosis of dermal melanocyte hamartoma was given. Dermal melanocytosis is clinically presented with dark blue-gray pigmentation and histopathologically shows increased number of ovoid to elongated melanocytes situated between collagen fibers in the dermis. There has been suggested a shared pathophysiologic mechanism between neurofibromatosis and other nevus in the context of organoid hamartoma. Some authors remarked to the concept of neurocristopathy while others suggested that fibroblasts in the dermis of neurofibroma produce growth factors influencing mast cells and melanocytes. Recent studies showed that hair follicle stem cells and melanocyte stem cells may act as niche structures for each other and Wnt/ß-catenin signaling pathway is a key factor in this connection. Genetic and larger case studies will provide the insight for why hairy pigmented patches and dermal melanocytosis are often found in neurofibromatosis patient.