Nevus of Ota, or nevus fuscoceruleus ophthalmomaxillaris, is a dermal melanocytic hamartoma that presents as bluish hyperpigmentation along the first or second branches of the trigeminal nerve. Extra-cutaneous involvement has been reported, especially ocular involvement. Nevus of Ota affects between 0.014%-0.034% of the Asian population. Clinical differential diagnoses include facial cafe-au-lait patch, spilus nevus, and acquired bilateral nevus of Ota-like macules. Previous treatment modalities, including cryotherapy and microsurgery, can be associated with scarring. In the last decade, the use of Q-switched lasers has revolutionized the treatment of this condition. This review summarizes the clinical, histologic, and management aspects of this dermal melanocytic hamartoma.