Pilomatrix carcinoma is a rare cutaneous tumor derived from follicular matrix with about 150 reported cases and it is considered as locally aggressive tumor with a tendency to recur. In addition to lymph node metastases and poor prognosis, it is non-chemotherapy responding in systemic metastasis.

A 37-year-old Libyan man presented with two large, coalesced nodules in the face measuring about 3*2 cm and 3*3 cm, treated as a case of lipoma by surgical excision based on clinical diagnosis, reappearing of larger nodule 9*6*4 cm involving almost all the left check, surgical excision was done with histopathological features of pilomatrix carcinoma infiltrating to the subcutaneous adipose tissue and deep striated muscle with no clear margins. Conventional radiotherapy by electron beam was started using the linear accelerating machine, with total radiotherapy dose 60 gray (Gy) in 30 fraction for six weeks. No local recurrences, nor lymph node or systemic metastasis since June 2020.

Pilomatrix carcinoma must always be considered in the differential diagnosis of nodular tumors of the head-and-neck due to high recurrence rate after simple excision. Furthermore, local radiotherapy post incomplete surgical excision is the best adjuvant therapy.