Proteus syndrome is a complex disorder characterized by a wide variety of deformities including macrodactyly. In the present report, we present a case with complex macrosyndactyly in his hand. The patient was surgically treated. However, he admitted with lymphangiomas on his body during the follow-up period, leading to the diagnosis of Proteus syndrome. This article discusses the differential diagnosis of Proteus syndrome and the treatment methods used for macrodactyly along with the presentation of the case.