To review and summarize the newest update on ocular sebaceous gland carcinoma (SGC) focusing on diagnosis and treatment. A PubMed search was carried out using the terms "Sebaceous Carcinoma", "Meibomian Gland Carcinoma", "Sebaceous Cell Carcinoma", and "Sebaceous Gland Carcinoma". All studies published in English up to October 2017 were included in this review. Globally, the overall incidence of SGC is increasing making it the third most common eyelid malignancy after basal cell carcinoma (BCC) and squamous cell carcinoma. The mainstay of treatment of ocular SGC is wide surgical resection under frozen section or Moh's micrographic surgery control followed by eyelid reconstruction. Based on histopathological features, SGC can be classified according to growth pattern, cell type, and cytoarchitecture. Based on the growth pattern, they can be classified as trabecular, lobular, papillary, and BCC-like. The cell type can be classified as basaloid, basosquamous, and epidermoid. The SGC cytoarchitecture presents either as a nodular or as an infiltrative lesion. Based on immunohistochemistry, the overexpression of ZEB2, BAG3, androgen receptor, and C-erbB-2 oncoprotein is associated with poor prognosis. The tumor is associated with systemic metastasis in 8-14% and death in 10-30%. Ocular SGC is an aggressive tumor associated with poor prognosis. Early identification and appropriate treatment may help improve the prognosis. New insight into its pathogenesis and the immunohistochemical profile may lead to the development of new effective treatment strategies, along with traditional therapies.