In this observation, we report an unusual presentation of a pilomatricoma in an 8-year-old girl who was initially referred to the department of vascular medicine for diagnosis and care of a suspected mixed lymphatic venous malformation. The lesion on her left shoulder presented as a giant bluish-purple red solitary mass, painful and rapidly growing, measuring 7 cm in anteroposterior diameter. This mass did not present the typical characteristics of a lymphatic venous malformation but exhibited warning signs of malignancy on clinical examination and imaging. The diagnosis of pilomatricoma was reached by fine-needle aspiration biopsy of the mass, showing mummified “ghost” squamous cells and a granulomatous inflammatory reaction stroma with scattered multinucleated giant cells and no sign of malignancy. Complete surgical excision associated with plastic surgery was curative. Diagnostic and management approach to skin lesion in childhood is subject of great concern for health care professionals, including dermatologists and vascular medicine specialists who may be confronted with this atypical presentation of one of the most common causes of superficial neck masses in children.