Extra Hepatic Portal Vein Obstruction in individual with solitary left kidney is rare occurence. Though there is no etiological association between Extra Hepatic Portal Vein Obstruction and solitary left kidney but the solitary left kidney decides the modality of treatment. Eighteen year lady referred to our institute with menorrhagia for 5 years and ultrasonography finding of splenomegaly and atretic right kidney. Investigations revealed Extra Hepatic Portal Vein Obstruction with multiple cavernoma formation with oesophagogastric varices with right renal agenesis. She successfully underwent splenectomy with devascularisation. Patient with Extra Hepatic Portal Vein Obstruction present mainly with recurrent episodes of variceal bleeding, splenomegaly and hypersplenism. Splenectomy and esophagogastric devascularisation is an effective modality of treatment for patient with Extra Hepatic Portal Vein Obstruction with solitary kidney. Keywords: cavernoma; modified Hassab's operation; Portal vein; unilateral renal agenesis.