Nevus comedonicus is an uncommon cutaneous developmental defect of follicular apparatus characterized by unilateral and linear distribution of bundles of dilated hair follicles filled with keratin plugs. It is usually seen on the head and neck region, trunk and upper arm. This condition may be present at birth or can occur later in life. The term nevus comedonicus is a misnomer as there are no true comedones and is better termed as follicular keratotic nevus. It is also known as nevus zoniforme or nevus acneiformis unilateralis. There are two types of nevus comedonicus, namely inflammatory and non-inflammatory (non-pyogenic). When nevus comedonicus is manifested with other extracutaneous symptoms, it is termed as nevus comedonicus syndrome. Diagnosis is mainly clinical, based on history and typical morphology. As the disease runs a benign course, no aggressive treatment is required. Patients seek treatment, especially for cosmetic purposes and inflammatory type of lesions. Various treatment modalities like topical therapy, surgical excision, lasers are available and treatment options are individualized based on the size and extent of the lesion. We herein present a case of unilateral nevus comedonicus with no systemic associations in a 30-year-old female on her lower limb which is not a common site of occurrence.