Aims: To determine the incidence and clinical features of patients diagnosed with pilomatrixoma. Patients and Method: A retrospective analysis was made of 205 cases of pilomatrixoma diagnosed according to clinical and histological criteria, with an evaluation of the incidence, patient age at presentation, gender, lesion location and size, single or multiple presentation, differential diagnosis, histopathological and clinical findings and relapses. Results: Pilomatrixoma was seen to account for 1.04% of all benign skin lesions. It tended to present in pediatric patients - almost 50% corresponding to individuals under 20 years of age - with a slight male predilection (107/98). Approximately 75% of all cases presented as single lesions measuring less than 15 mm in diameter. Multiple presentations were seen in 2.43% of cases. The most frequent locations were the head and orofacial zones (particularly the parotid region), with over 50% of all cases, followed by the upper (23.9%) and lower limbs (12.7%). Only one relapse was documented following simple lesion excision. Conclusions: The frequency of pilomatrixomas was 1.04% of all benign skin lesions - the lesions being predominantly located in the maxillofacial area. Due to the benign features of this disorder, simple removal of the lesion is considered to be the treatment of choice, and is associated with a very low relapse rate. © Medicina Oral S. L.