Verrucous epidermal nevi are noninfl ammatory, congenital, cutaneous hamartomas composed of keratinocytes, abnormal clone(s) of cells that refl ect genetic mosaicism arising from different somatic mutations. Some of these mutations are well recognized, but some are still unidentifi ed. Molecular techniques are used for identifi cation and classifi cation of molecular causes of certain epidermal nevi, whereas all verrucous epidermal nevi are divided into epidermolytic and non-epidermolytic types. They are typically present at birth, but may appear during childhood, even later. Their prevalence in adults ranges from 0.1 to 0.5%, equally affecting both sexes, and about 1 in 1000 newborns. Warty, brown papules without infl ammation distinguish verrucous nevi from other epidermal nevi, while presence at birth and persistance distinguish verrucous epidermal nevi from linear viral warts. Epidermolytic and non-epidermolytic verrucous epidermal nevi are almost always hard to distinguish, except by histology. As a rule, verrucous epidermal nevi are asymptomatic, they have a benign course, except occasionally, and therapy is mostly used for cosmetic reasons. Simple excision is usually the treatment of choice. Topical agents are rarely curative, as well as surgery which is associated with relapses, unless both epidermis and the underlying dermis are removed or destroyed at the same time. We present a case of an otherwise healthy 21-year-old female patient, who presented with a solitary congenital verrucous caulifl ower-like lesion in the right zygomatic region of the face. The lesion was present from birth. Due to its gradual growth during years, the lesion became a great esthetic and functional problem for this young patient. There was no family history of similar or any other tumorous skin lesions in the family. On examination, the patient had a solitar unilateral, well defi ned yellowish caulifl ower-like verrucous lesion confi ned to the right malar side of the face. The lesion was distributed along the lines of Blaschko extending horizontally, from its wider 1.5 cm caulifl ower-like part on the right zygomatic region, towards its tail-like 0.5 cm thick end on the preauricular region, in approximately 3 cm long tail-like manner without crossing the midline. Since the patient refused biopsy, no exact differentiation between epidermolytic and non-epidermolytic nevi was possible. The diagnosis of verrucous epidermal nevus was based on history and clinical presentation, as a diagnosis of exclusion. Due to the fact that patients with epidermolytic verrucous epidermal nevi are at risk of parenting a child with bullous ichthyosiform erythroderma, the patient was counseled on this risk, and on the possibility of fi rst-trimester antenatal diagnosis. The lesion was successfully treated by radio-wave surgery.