We report a case of congenital organoid nevus with an excessive number of apocrine glands. A one-year-old boy presented with a slowly enlarging keratotic element in the left temporal region, and biopsy showed hyperkeratosis, acanthosis, follicular plugging and multiple transverse sections of mature apocrine glands filling the reticular dermis. The lesion was benign and treatment was therefore conservative. In rare cases, various types of adnexal neoplasms may develop, but malignant tumours occur very seldom.