Introduction: Hopf's verruciform acrokeratosis is a genodermatosis characterized by keratotic lesions on the dorsa of the hands and feet. It usually develops during childhood but can occasionally develop at later ages, even in the fifth decade. Case report: We present the case of a 34-year-old man with phototype IV, who presented with cutaneous lesions that affected the forearms and hands on the dorsal surface, with a tendency to symmetry, consisting of multiple skin-coloured keratotic papules and erythematous lesions, of different sizes, some with a tendency to converge, rough surface with well-defined edges. The lesions were characterized by a chronic evolution. Conclusions: Because of its low prevalence, there are still no treatment guidelines, however topical or oral retinoid treatment, as well as laser and cryotherapy, are useful. The disease is prone to recurrence.