Desmoplastic melanoma (DM) is a rare and histopathologically as well as prognostically distinct subset of melanoma that arises in chronically sun-damaged skin. DM is typically and relatively indolent in nature with most cases not progressing to metastatic disease. DM has been managed with both radiation and surgical approaches. Medical options for the treatment of metastatic DM have traditionally been limited. Recent advances in immunotherapies have shown promising responses in DM ushering in a new class of treatment options. Additionally, with the advent of whole exome sequencing, the genetic make-up of DM has been further characterized creating new possibilities for future targeted therapies.