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Volvulus of the spleen is a complication of a previous congenital anomaly consisting basically in the absence of supporting structures of the spleen. Clinically, it is an extremely rare complication. It may present as an acute abdomen. The search of the literature that we conducted does not show any such case being the cause of portal hypertension. This motivated this case report. The patient was treated surgically and this cured the patient.
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