Adult T-cell leukemia-lymphoma (ATLL) is a malignancy of mature T lymphocytes caused by chronic human T-lymphotropic virus, type I (HTLV-I) infection. Up to one third of cases of ATLL can present with skin involvement-oftentimes there may only be skin involvement. Rare cutaneous presentations can further obscure the diagnosis, create diagnostic dilemma, and delay the institution of appropriate therapy. We present a case of ATLL where the initial lesion at presentation was pyoderma gangrenosum (PG). To our knowledge, there are no reported cases of ATLL presenting as PG.