McKittrick-Wheelock syndrome is a rare disease characterized by a large hypersecretory rectosigmoid villous adenoma resulting in persistent large volume diarrhea, electrolyte abnormalities, and renal dysfunction. We report an unusual presentation of this syndrome in a patient who developed persistent diarrhea along with dermatomyositis and rectal prolapse and was later discovered to have a large rectal villous adenoma along with a smaller sigmoid tubulovillous adenoma. In our literature review, we were able to find one case report of a dermatomyositis occurring in conjunction with a tubulovillous adenoma and few case reports of rectal prolapse in the setting of a secretory villous adenoma. However, there were no reports on both occurring in association with McKittrick-Wheelock syndrome. This report highlights the variable manifestations of colorectal adenomas and the importance of searching for an underlying neoplastic entity in patients with new onset dermatomyositis or rectal prolapse or both.