Abstract
Pilomatrixoma (PMX), also known as calcifying epithelioma of Malherbe, is a rare benign neoplasm that arises from the hair matrix cells, commonly in the head, neck, and upper trunk regions, infrequently affecting upper and lower extremities. It has to two peaks of presentation: under 20 years of age or between 50 and 65 years of age, slightly more common in females. The neoplasm exhibits diverse clinical manifestations and is frequently subject to misdiagnosis with alternative dermatological diseases. We present an atypical case of PMX affecting the upper extremity of a 62-year-old female patient. Surgical removal of the affected tissue under local anesthesia was performed, and subsequent histopathological analysis confirmed the presence of PMX. Based on the literature search we performed, we found out that this pathology is underreported in Jordan, with only one study published describing this tumor in the maxillofacial region. Physicians should be aware of this condition and its different presentations to include it in the differential diagnosis of suspected cases to provide the appropriate management and follow-up.