Epidermodisplasia verruciformis (EV) is a rare, inherited disease. The disease occurs with human papilloma virus (HPV), which has more than 70 types. Patients have a high risk of developing skin malignancies on sun-exposed areas. Ichthyosis vulgaris is an autosomal dominant inherited skin disorder and characterized by diffuse scaling. Becker nevus is a benign cutaneous hamartoma that presents as both hypermelanosis and hypertrichosis. Nevus spilus is an epidermal nevus consisting of a light tan patch with numerous dark brown macules and papules. Coincidence of some of the dermatological diseases were reported in the literature. However, the concurrent occurrence of EV with ichthyosis vulgaris, Becker nevus and nevus spilus has not been previously reported. Herein, we present a 36 years old male diagnosed with four dermatological diseases: EV, ichthyosis vulgaris, nevus spilus and Becker nevus.