Cutaneous paraneoplastic syndromes play an important role in the detection of malignant neoplasms and may represent the first clinical sign of an underlying malignancy. The skin is the second most frequently involved organ, after the endocrine system and endocrine syndromes, capable of indicating the presence of cancer. Malignant neoplasms, regardless of their type, constitute one of the leading causes of death worldwide. Their early diagnosis is a key component of oncological strategy. Early identification of paraneoplastic syndromes is of critical importance, as it accelerates the recognition of the malignancy and the initiation of effective treatment, thereby increasing the patient’s chances of survival. The aim of this publication is to present information on the occurrence, pathomechanisms, clinical course, and treatment of cutaneous paraneoplastic syndromes.