Introduction:

Tufting enteropathy, also known as intestinal epithelial dysplasia, is a very infrequent congenital disorder presenting as refractory diarrhea in infants.

Objective:

Describe the first report of congenital tufting enteropathy in Cuba.

Case presentation:

A presentation is provided of the first case of the disease in Cuba based on histopathological findings and accompanied by a description of the clinical, diagnostic and therapeutic aspects addressed.

Conclusions:

Tufted enteropathy poses a diagnostic challenge as it does not exhibit a pathognomonic clinical courtship. The concomitance of chronic diarrhea with malformation disorders should set off alarms and guide clinical thinking and diagnostic methodology towards possible genetic disorders.

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