Mucoepidermoid carcinoma (MEC) is most commonly found to affect the salivary glands and rarely affects other organ systems. In this report, we present an especially rare case of cutaneous MEC affecting the eyelid. A 72-years-old female patient presented with a painless, cyst-like, progressively enlarging lesion of the left upper eyelid. Biopsy revealed characteristic features of MEC, including a mixture of mucus-secreting cells and epidermoid cells. The 3 mm MEC lesion was excised by Mohs micrographic surgery, leaving a 0.8 × 1.1 cm eyelid defect that was repaired by oculoplastic reconstruction. To date, the patient has been in remission for 4 years with good functional and aesthetic outcomes. This case represents the longest reported remission among only 9 previously reported cases of cutaneous MEC of the eyelid and highlights the therapeutic and aesthetic efficacy of Mohs micrographic surgery. We also offer a literature review of the 9 previously reported cases of eyelid MEC and a discussion of patient presentation, treatment comparisons, remission success, and key points in the management of MEC of the eyelid.