Epidermal nevi are hamartomatous lesions that are typically present at birth, but can occur anytime during childhood and may rarely appear in adulthood. An estimated one-third of individuals with epidermal nevi have involvement of other organ systems; hence, this condition is considered to be an epidermal nevus syndrome. There are four distinct epidermal nevus syndromes recognizable by the different types of associated epithelial nevi: linear sebaceous nevi, linear nevus comedonicus, linear epidermal nevus, and inflammatory linear verrucous epidermal nevus (ILVEN). Each type may be regarded as a part of a syndrome with other systemic manifestations. We report a rare case of ILVEN syndrome in a 23-year-old female patient with a wide spectrum of mucosal, cutaneous, and skeletal abnormalities, demonstrating the polymorphic presentation of this condition.