Skin adnexal carcinomas (SACs) represent a diverse range of cancerous growths originating from the appendages of the skin. SACs are exceedingly rare malignancies that primarily manifest in individuals with fair skin and predominantly affect the head and neck. In the present study, a 70‑year‑old male presented with swelling and redness around the right eye, accompanied by skin desquamation. A tissue biopsy from the lesion showed a poorly differentiated SAC. Magnetic resonance imaging (MRI) of the neck and base of the skull revealed the presence of a large, well‑defined heterogeneous mass involving the right orbit and periorbital tissue, measuring 62x38x43 mm. The patient declined wide local excision of the tumor. Eventually, the patient received a combination of chemotherapy and radiotherapy, followed by additional cycles of chemotherapy. Then, 1 month after the final chemotherapy, an MRI showed a small, linear lesion (8x4 mm) in the lower right orbital cavity, reduced by 90% from its original size. The scan also revealed diffuse volume loss and irregularity in the right eye, likely due to radiation, leading to vision loss in the affected eye. At present, the exact cause of SACs remains largely unidentified. While SACs frequently develop spontaneously, some may originate from precursor lesions or preexisting benign counterparts. The incidence of SAC is exceedingly rare, particularly when it is found in the eyelid. A combination of chemotherapy and radiotherapy followed by additional cycles of chemotherapy may be an effective therapeutic modality in shrinking the tumor.

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