Karyomegalic interstitial nephritis is a rare cause of hereditary interstitial nephritis. There are few case reports of Ifosfamide, an alkylating agent causing karyomegalic interstitial nephritis. There are 4 case reports described in children and adolescents. There is only one case report described in adults. Here we present a case report of a 36 year old gentleman, who was treated with ifosfomide as part of chemotherapy regimen for myxoid liposarcoma. He later went on to develop renal dysfunction requiring renal replacement therapy. Evaluation of renal dysfunction with Renal biopsy revealed karyomegalic interstitial nephritis. He had significant improvement with steroid treatment. He was weaned off hemodialysis. This emphasizes the importance of diagnosis of this condition and appropriate therapy with steroids for reversal of renal dysfunction.