A case of intestinal lipodystrophy (Whipple's disease) was reported in a Negress—the first published case in the Negro race. The diagnosis was made by transoral jejunal biopsy with an intestinal biopsy (Crosby) capsule and was later confirmed by postmortem examination. Microscopic examination of the jejunum revealed histiocytes containing foamy cytoplasm which was periodic-acid-Schiff (PAS) positive. Sickle-form particles were seen in the jejunum, liver, spleen, kidneys, urinary bladder, ovary, and lymph nodes. These findings are considered to be pathognomonic of intestinal lipodystrophy and reveal the systemic nature of the disease. The frequent association of the disease with tuberculosis calls for further investigation. Because of the lymph node involvement, differentiation from sarcoidosis and malignant lymphoma is necessary.