Objective: To analyze the clinicopathological features of PMC, including the patient age at clinical presentation, gender, anatomical location, tumor size, preoperative clinical and histopathologic diagnosis. Methods: This is a retrospective study to analyze all cases of PMC diagnosed in two hospitals from August 2008 to January 2017 in Southern Region, Saudi Arabia. Detailed medical records and histopathologic material review were done for all cases of pilomatricoma. Cases were identified using the search engine of each hospital electronic filing system. Results: 23 cases with an age range from 8 to 68 years (mean 28.8). The majority of patients were in their second (39%) and fourth (26%) decades of life with a female to male ratio of 1.2 to 1.1. Majority of the cases were noted in the neck (43.5%), followed by head region (30%). The tumor size ranged from 0.3 to 4.0 cm (mean 1.6). The most common preoperative clinical misdiagnosis was sebaceous cyst (34.8%). All cases were confirmed histopathologically as pilomatricoma. All tumors were excised surgically. Conclusions: PMC has a wide variety of clinical presentations and is commonly misdiagnosed with other dermatological conditions. PMC should be included in the differential diagnosis of nodules, particularly in the head and neck region. Increasing the clinical awareness and familiarity with the tumor may lead to accurate clinical diagnosis.