Primary cystic adenoid skin carcinoma is a rare and poorly documented neoplasm in literature worldwide, with just over 250 reports. This work describes a 52-year-old male patient, with no comorbidities, who presented this neoplasm in nodular format in the posterior thoracic region, associated with localized pain and erythema — symptoms that led him to seek medical help. The clinical findings, differential diagnosis and treatment particularities were reviewed and correlated with the clinical case. The choice of type of surgical treatment was done considering the characteristics of the primary lesion that are associated with a worse prognosis. Despite its rarity, this neoplasm is easily identified through histological examination, the correct choice of treatment and patient follow-up, essential to increase survival. Thus, this work contributes to diminish the scarcity of literature related to this topic, especially the form of treatment employed.