Sir, Epidermal nevi are asymptomatic lesions present since birth or appearing within the 1st year of life. They are usually stable and do not require any treatment.[1] However, reports of benign and malignant changes occurring over these lesions have been reported, such as psoriasis, keratoacanthoma, squamous cell carcinoma, and basal cell carcinoma.[234567] To the best of our knowledge, there have only been four case reports of keratoacanthoma developing over a preexisting epidermal nevus, and only one case from India.[1567] A 47-year-old male presented with a single asymptomatic dark-raised lesion over the left forehead since birth. He developed an asymptomatic growth over this lesion for 6 months, which progressively increased to its present size. It was not associated with pain, oozing, bleeding, or ulceration. There was no history of recent change of color of the lesion. There was no history of preceding trauma. There was no family history of similar complaints. He had a history of taking treatment for pulmonary tuberculosis 5 years ago. Cutaneous examination showed a single, well-defined hyperpigmented plaque measuring about 4 cm × 3 cm with a verrucous surface over the left temple, above the lateral canthus. A single exophytic hyperpigmented growth was present over the inferior aspect of the above lesion [Figure 1]. Systemic examination did not reveal any abnormality. Blood and urine routine investigations were within normal limits. The lesions were removed by radiofrequency ablation. Histopathological examination of the plaque showed mild hyperkeratosis, irregular acanthosis, flat, broad papillomatosis, focal hypergranulosis, and increase in basal melanin pigment. Dermis showed congested ectatic blood vessels and dense perivascular and perifollicular lymphocytic infiltrate. These features were in favor of epidermal nevus [Figures 2 and 3]. The overlying growth on histopathological examination showed a large hyperkeratotic plug overlying multiple, discrete, dilated, and keratin-filled follicular infundibula, with irregular acanthotic growth from the base of the infundibula into the base of the epidermis. There was no evidence of dysplasia or malignancy. These features were consistent with keratoacanthoma [Figures 4 and 5]. Based on the above findings, a final diagnosis of keratoacanthoma overlying an epidermal nevus was made.Figure 1: A single, well-defined hyperpigmented, verrucous plaque above the left lateral canthus, with an exophytic hyperpigmented growth over its inferior aspectFigure 2: Section of the plaque showing hyperkeratosis, irregular acanthosis, flat, broad papillomatosis, focal hypergranulosis, and increase in basal melanin pigment (H and E, ×10)Figure 3: Section showing numerous ectatic blood vessels in the dermis, along with perivascular and periadnexal infiltrate (H and E, ×10)Figure 4: Section of the growth showing a large, central hyperkeratotic plug overlying multiple discrete, dilated, keratin-filled follicular infundibula (H and E, ×10)Figure 5: Section of the growth showing irregular acanthotic epidermal downgrowth from the base of the dilated infundibula, along with numerous ectatic vessels in the dermis (H and E, ×10)Epidermal nevi are the most common type of keratinocytic nevi.[1] These are asymptomatic lesions present since birth or appearing within the 1st year of life.[2] Rarely, they may develop even in adolescence.[1] They may increase in size till adolescence, after which they usually remain stable. They do not require any treatment, except for cosmetic purposes, although they may rarely be complicated by secondary infection, maceration, or neoplastic changes.[12] Recently, reports of benign and malignant changes occurring over these lesions have been noted, including the development of psoriasis, keratoacanthoma, squamous cell carcinoma, and basal cell carcinoma.[34567] Malignant transformation is more likely to occur in the middle and elderly age groups.[2] To the best of our knowledge, there have only been four case reports of keratoacanthoma developing over a preexisting epidermal nevus and only one case from India.[2678] We present the above case in view of the rarity of its occurrence. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.