Clear cell acanthoma (CCA), is an asymptomatic benign lesion of unknown origin and aetiology, which typically presents as a red to brown, dome-shaped papule on the leg. We discuss the case of a patient with an irregular plaque of hypopigmented papules whose diagnosis of CCA was only made by biopsy, and review the characteristic presentation, uncommon variants, locations and associated conditions of this lesion. The diagnosis is based on histopathology and/or immunohistochemistry, but the variable clinical presentation may make diagnosis difficult. The differential diagnosis includes skin tumours and inflammatory and pigmentary dermatoses. The lesions do not regress spontaneously, and excisional removal is the preferred treatment.