We report two cases of microvillous inclusion disease (MID) occurring in a set of siblings. Although it is a rare disorder, MID appears to be a common cause of familial intractable secretory diarrhea. Diagnosis rests on the ultrastructural finding of intracytoplasmic inclusions that are lined by intact microvilli. These inclusions are present in the absorptive surface epithelial cells of the small and large intestine and are associated with poorly developed surface brush border microvilli. The prognosis of MID is poor and curative therapy is not currently available. Because MID appears to be a hereditary disorder, genetic counseling of affected families is essential.