Pilomatricomas are benign neoplasms of the hair follicle occurring relatively often in the pediatric population. However, lesions attaining massive proportions are very rarely encountered. We describe such a case presenting in the parotid region and initially misdiagnosed as malignancy. Preoperative biopsy provided accurate diagnosis and allowed definitive surgical excision using a parotid-sparing approach. In children, giant pilomatricoma should be included into the differential diagnosis of noninflammatory masses of the salivary gland region. (C) 2011 Elsevier Inc. All rights reserved.