A 65-year-old female with celiac disease developed cholestatic jaundice and fatal liver failure. Investigations revealed widespread necrotic foci in the liver, spleen and mesenteric lymph nodes, changes reminiscent of the mesenteric lymph node cavitation syndrome, which is known to complicate celiac disease. In addition, malignant lymphoid cells were present infiltrating hepatic sinusoids, lymph nodes and spleen. These features are typical of hepatosplenic lymphoma, a rare type of peripheral T cell lymphoma with T cell receptor rearrangement. Lymphorecticular malignancy complicating celiac disease may present with fulminant liver disease.