Background: Pilomatricoma, the most common hair follicle tumor, has a wide variety of clinicopathological characteristics and is often misdiagnosed. Objective: The aim of this study was to explain the clinical and histological spectrum of tumor in order to help for its better diagnosis. Patients and Methods: In a retrospective descriptive study, the clinical and histological features of pilomatricoma were reviewed. The files and histologic sections of all biopsy specimens examined in Hamadan Sina Hospital between 1992 and 1999 were analyzed. Results: Among 4000 biopsy specimens, 22 cases of pilomatricoma were found. 59.3% of the lesions were located on upper extremities. The lesion developed in second decade of life in 36.5 percent of the patients. Shadow cells, calcifications, ossification and granulomatous reaction were detected in 100, 50, 9 and 45.5 percent of specimens, respectively. The accuracy of clinical diagnosis compared with pathologic findings was 36.5 percent. Conclusion: Pilomatricoma was the final diagnosis in 0.55 percent of all biopsy specimens examined in Hamadan Sina Hospital between the years 1992 to 1999. It should be included in differential diagnosis of skin tumors.