In this review the normal histology of the skin adnexal structures is reviewed, and the classification of the benign and malignant tumors and tumor-like lesions of pilosebaceous origin is given. Pilomatrixoma or Malherbe’s calcifying epithelioma is the most common type of pilar tumors. It is a benign neoplasm that arises from hair follicle matrix cells. The tumor can cause diagnostic difficulty not only for the clinician but also for the pathologist. Also clinical and histopathological features of pilomatrixoma is reviewed with literature.