OBJECTIVE: To present a rare case of pilomatrixoma involving half of the face of a six-year old child. CASE REPORT: A six-year-old, black, male child was brought with a lesion in the left hemi-face; the lesion had arisen two months ago and had been growing steadily ever since. External examination showed a child, overall in good condition, with a single nodular, elastic, mobile and slightly tender lesion covered with skin, in the left hemiface. The lesion was removed on surgery after incision of the overlying skin, and sent for histopathological examination, resulting in a diagnosis of Malherbe's Calcifying Epithelioma. CONCLUSION: The clinical presentation of this case is consistent with that described in the literature, i.e., a benign tumor usually occurring in childhood, presenting as a single mobile subcutaneous nodule of elastic consistency, generally in the face, neck and upper limbs. There is a predilection for the white race and the female sex, none of which was the case in our patient. It is considered a rare case by some authors, and only apparently rare by others because of misdiagnosis. It is difficult to diagnose clinically, and requires an excision biopsy for histopathological identification. The differential diagnosis includes several clinically similar lesions such as hemangiomas, dermatofibroma, and basal and squamous cell carcinomas, among others. The treatment is always by surgical excision. Recurrence is rare, as is malignant transformation; however, it is important to follow up these patients.