Abstract
Introduction: The incidence of Pilonidal Sinus Disease (PSD) is increasing worldwide, especially in the developing and developed countries. As long-standing chronic infection may trigger neoplastic transformation, more carcinoma arising from PSD are to be expected with an incidence of 0,1%.
 Methods: Case report describing a Squamous cell carcinoma (SCC) arising from PSD
 Results: A 60-year-old male presented with a 6cm x 10cm ulcerating wound in the sacrococcygeal area, which was painful for 3 months. A biopsy initially revealed a highly differentiated squamous cell carcinoma (cT3cN1acM0G1). Soft tissue MRI showed possible infiltration of the coccyx, and a staging CT showed suspicious enlarged lymph nodes within both inguinal regions. The patient underwent a full 20x1,8 Gy chemoradiation with partial response of the primary. Surgical Resection was recommended. The patient did not show up for follow-up after chemoradiation and refused further treatment. He demised after 14 months due to pulmonary metastases.
 Conclusion: Even a curative intended chemoradiation of this highly differentiated tumour led to a dismal outcome of disease after 14 months. Cases of PSD carcinomas often present as atypical cutaneous SCC.
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