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A case of Peutz-Jeghers syndrome (PJS) in an African girl is presented to document the clinical presentation, management and follow up of this condition. The patient who presented with black buccal mucosal hyperpigmentation and clinical features of jejuno-jejunal intussusception was successfully managed by operative reduction of the intussusception and polypectomy. She is being followed up for evidence of malignant transformation in associated intestinal polyps and development of extra-intestinal malignancies at other sites. The management of 1'JS in light of recent trends is discussed, especially with reference to suggested protocols for screening and surveillance of sites at supposed risk of tumour development.East African Medical Journal, May 1999, 284-286