A rare malignancy, pilomatrix carcinoma (PC) histologically originates from matrix cells in skin hair follicles. Pathologically, it can be confused with squamous cell carcinoma (SCC). A 56-year-old male was referred to us with a diagnosis of SCC of the right lung. After initial histopathological evaluation, the lung tissue was re-examined immunohistochemically, and a diagnosis of metastatic PC was made. Due to an absence of standardized treatment for metastatic PC, we administered a combination of carboplatin and paclitaxel due to its wide-spectrum anti-tumoral activities; however, the disease progressed despite first-line treatment. Although cyclophosphamide and etoposide were administered orally as a second-line therapy, the patient showed little progress and passed away despite two chemotherapy courses. Hence, the diagnosis, treatment, and prognosis of this rare tumor present several challenges. We aimed to contribute to this existing dilemma with our case report and literature review.