Enzinger and Weiss first described Collagenosis Nuchae (CN) or nuchal fibroma (NF) in 1988. CN is a rare benign soft tissue tumour that arises from the posterior cervical subcutaneous tissue with predilection for the interscapular and paraspinal regions. It is more common in males. CN has been reported in association with trauma, diabetes, scleredema and Gardner's syndrome. The true incidence of CN is probably higher than recognised and CN should be in the differential diagnosis of head and neck lesions. Histological examination is required for the diagnosis. Careful total excision provides cure and accurate diagnosis. The purpose of this study is to review the epidemiological, clinical, histopathological and radiological features of this rare lesion, which is often misdiagnosed.