This brief literature review is dedicated to the history of the disease and the term “black acanthosis,” its etiology, pathogenesis, classification, clinical picture, diagnosis, and treatment of this pathology, as well as a description of a clinical case of an adult patient with black acanthosis. Black acanthosis refers to a heterogeneous group of warty keratoses characterized by villous-warty growths and skin hyperpigmentation. Currently, five clinical forms of black acanthosis are distinguished: pseudoacanthosis, hereditary benign, drug-induced, benign, and malignant. The benign form of black acanthosis is associated with endocrine disorders, particularly with insulin resistance and diabetes mellitus. The malignant form represents a paraneoplastic process and more frequently arises against the background of adenocarcinomas of the abdominal organs and cancers of the urinary and reproductive systems and serves as a poor prognostic factor. Black acanthosis may be associated with a paraneoplastic syndrome causing palmar scarring. Since black acanthosis is considered a dermatological manifestation of somatic pathology, its treatment is symptomatic and involves managing the underlying disease. The presented clinical case of malignant black acanthosis illustrates the need for comprehensive clinical, laboratory, and instrumental examinations of patients to exclude or identify oncological pathology early on.