Background: Pilomatrixoma (benign calcifying epithelioma of Malherbe) is an uncommon benign tumor. It is frequently misdiagnosed as epidermoid or dermoid cyst. It has certain characteristic clinical and histopathologic features, but since it is not commonly suspected preoperatively, certain distinctive clinical features of tumor may suggest clinical diagnosis followed by histopathologic confirmation. We presented a female patient with pilomatrixoma who was treated many times with the preliminary diagnosis of lymphadenopathy. Case presentation: We presented a case of a 15-year-old female found to have a large left cervical mass (6 cm×2.5 cm) and regional lymphadenopathy (<0.5 cm). The patient had a painless, slowly growing lesion over the left cervical region of 4 months duration. Examination showed a localized, subcutaneous, well-circumscribed, non-tender, pinkish-reddish mass with yellowish peripheral discoloration. The mass was firm-to-hard and adherent to overlying intact skin with gritty surface with telangiectatic vessels on palpation. Other physical examination findings and laboratory examinations were normal. MRI revealed a heterogeneously hyperechoic superficial oval formation with hypoechoic halo and calcifications. The mass was biopsied and proven to be a pilomatrixoma. Conclusion: More than 70% of pilomatrixoma originate in the head and neck. The main pathological conditions of the neck in pediatric age are thyroid dysgenesis, thyroiditis, thyroid nodules, lymphadenopathies, cystic lesions, hemangiomas, vascular malformation, cervical thymus, fibromatosis colli and pilomatrixoma. Pilomatrixoma may rarely undergo malignant transformation into pilomatrix carcinoma. Unless the lesion is symptomatic, no treatment is required