To report a rare case of a painless mass on the lower lid, histologically diagnosed as primary signet ring cell carcinoma of the eyelid.

A 74-year-old male presented with a painless mass on the right lower lid which had developed seven months prior to presentation. Incisional biopsy of the mass and attached lower lid skin was performed, revealing signet ring cell carcinoma. A systemic evaluation, including whole body positron emission tomography-computed tomography (PET-CT), chest CT, abdomen and pelvis CT, gastrointestinal endoscopy, and colonoscopy, revealed no other abnormal lesions. Therefore, the eyelid lesion was considered primary signet ring cell carcinoma of the skin and was treated with radiotherapy of 6600 cGy in 33 fractions over 7 weeks.

Herein, the authors report a rare case of primary signet ring cell carcinoma of the eyelid with no evidence of tumor recurrence or metastasis for 5 years after radiotherapy.