In 1898 Banti described a disorder characterized by splenomegaly and hypersplenism, resulting in portal hypertension and anemia in the absence of hematological disease. It usually occurs around 3rd to 4th decade of life. It is known as non-cirrhotic portal hypertension (NCPH) in India and Idiopathic Portal Hypertension (IPH) in Japan. Hepatoportal sclerosis seems to be its counterpart in the United States. Banti's syndrome is a disorder of unknown etiology. It has been reported from Indian subcontinent. We report a case of atypical presentation Banti's syndrome in 34-year old man presenting to us with perianal and scrotal swelling. He was subsequently diagnosed to have Banti’s syndrome.