With reference to an observation, thirty-four cases of systemic mastocytosis with intestinal malabsorption are reviewed. The most characteristic intestinal anomalies are the steatorrhea, partial villous atrophy and deficiency in secretory IgA. Routine investigations for mastocytosis may be warranted in patients with malabsorption. A markedly telangiectatic and angiomatous aspect of cutaneous lesions may be suggestive of the association.