Leukemia cutis is the infiltration of the skin by malignant myeloid cells, most commonly due to underlying acute myeloid leukemia (AML). Leukemia cutis can predate the manifestation of systemic leukemia (aleukemic leukemia cutis) or develop concurrently with bone marrow involvement (synchronous leukemia cutis). Patients with aleukemic leukemia cutis invariably develop systemic disease. Leukemia cutis may be the first sign of disease relapse after allogeneic hematopoietic stem cell transplant. Clinical manifestations of leukemia cutis are protean and comprise a wide range of skin lesions, from mild macular rash to ulcerated tumors. The diagnosis should be made using clinicalopathological correlation. Histology alone is often non-specific or misleading, simulating many benign and malignant skin diseases. Diagnosis needs to be informed by ancillary techniques, such as immunohistochemistry. There may be discordance of phenotype and cytogenetic aberrations in leukemia cutis relative to leukemic cells in bone marrow. No consensus management guidelines exist, although systemic treatment is usually required. Radiotherapy may be an effective skin-directed palliative therapy.