An 18-year-old male presented with an erythematous plaque on the left side of face and neck since 4 years. The lesion was devoid of hair, had follicular prominences, and showed increased localized sweating. Histopathology revealed an increased number of eccrine glands in the myxoid stroma with multiple vascular elements in the dermis, favoring the diagnosis of eccrine angiomatous hamartoma. This case illustrates a relatively uncommon clinical presentation of eccrine angiomatous hamartoma as a hairless, hyperhidrotic plaque in the head and neck region.