The clinical presentation of Dupuytren's disease is discussed with emphasis on dermato-pathology, the nodule, the cord, ectopic manifestations regional and distant, and disease progression. The differential diagnosis also is described with a list of pseudo-Dupuytren's disease cases. Observations by this author suggest that there are two distinct clinical entities responsible for palmar fascial contracture, namely typical Dupuytren's disease and atypical Dupuytren's contracture. These two types seem to differ in presentation, treatment, and prognosis. The characteristic clinical findings of each of these two types are described. The disparity among treatment outcome studies and epidemiologic studies with regard to the prevalence of Dupuytren's disease is probably in part due to lack of distinction between these two clinical types. Accurate diagnosis and satisfactory treatment outcome can be achieved by careful history, thorough physical examination, and keen understanding of the pathophysiology of this enigmatic disease.