Pilonidal disease is a chronic disorder which is observed in boys and men between ages of 15 and 40 years old in Caucasian race. Malignant degeneration and carcinoma arising from chronic sacrococcygeal pilonidal disease are observed extremely rare and are described in the group of Marjolin cancer. Carcinoma can be seen on a place with long-term inflammation process as it was in the case described in the patient’s article. First time squamous cell carcinoma arising from chronic sacrococcygeal pilonidal disease was described in 1900 by Wolff. The incidence of carcinoma arising in pilonidal disease is less than 0,1% and in the whole medical journals less than 100 patients are reported. This article presents the case of a patient with diagnosed squamous cell carcinoma arising from chronic sacrococcygeal pilonidal disease. In this article was presented the clinical features, pathogenesis, treatment options and prognosis of this rare disease.