A 47-year-old female was referred to our department because of skin-colored lesions located mainly on the dorsum of the hands and feet. The patient had no diseases and had not taken continuous medication. The lesions were present at birth and had increased in number since then. Four more members of the family, including the mother and the uncle (mother’s brother), had similar skin lesions. A dermatological examination revealed multiple flesh-colored, flat-topped papules, about 2–5 mm in size, which were reminiscent of flat warts on the dorsum of the hands and feet (Fig. 1). A nail examination revealed thickened nails with V-shaped nicks at their free margins on the fingers of both hands (Fig. 2). The patient declined skin biopsy. We retained the diagnosis of acrokeratosis verruciformis of Hopf (AVH). AVH is a rare inherited disorder of keratinization caused by an abnormal ATP2A2 gene [1,2]. Familial cases indicate an autosomal dominant pattern of inheritance, yet sporadic cases of the disease have also been reported [2]. It is characterized by multiple asymptomatic, flat-topped, skin-colored papules on the dorsum of the hands and feet. Other features may include pits on the palms and soles and nail dystrophy (pearly white nails or nails with longitudinal ridges and nicks in the free edge) [3]. AVH is diagnosed clinically, which becomes easier if there are family members with similar skin findings. Sometimes, a skin biopsy is performed to assist the diagnosis. The key features on histology are hyperkeratosis, hypergranulosis, acanthosis, papillomatosis, and circumscribed epidermal elevations known as church spires, which are a distinctive finding [1,2].