We report herein a rare case of isolated collagenoma located on the plantar surface with macrodactyly of the 3rd and 4th toes occurring on the right foot, which is considered as an incomplete form of Proteus syndrome.

A 36-year-old male with normal intellectual and physical development visited with a pedunculated, soft, dome-shaped tumor and multiple, grouped, slightly elevated nodules on the right plantar region and hypertrophy of the 3rd and 4th toes of the right foot. The lesions appeared during early childhood and have been growing steadily since then. Total excision of the plantar mass with skin grafting and debulking of the 3rd toe and amputation of the 4th toe were done.