| Summary: | Abstract Pilomatrixoma is a benign hair follicle tumour. Anetodermic changes overlying pilomatrixoma are rare. The aim of this study is to evaluate a case series of patients with a clinical diagnosis of anetodermic pilomatrixoma presenting to our Dermatology Department over a 5‐year period. Eight cases were identified. The median age of onset was 21 years. All cases presented on the upper limbs and trunk with a solitary rapidly evolving tumour, tender on palpation. They had an erythematous protuberant appearance with a wrinkled and atrophic surface. Underlying pilomatrixomas were firm measuring 1–5 cm. Simple excision was carried out in seven cases without postoperative complications. In conclusion, anetodermic pilomatrixoma is a rare variant of this tumour, occurring more frequently on the upper body. It presents with identifiable features and should be differentiated from other skin tumours. Surgical removal is usually the gold standard treatment.
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