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A 4-year-old boy presented with severe bone pains, refusal to walk, diffuse bony swelling of forelimbs, skin changes and abdominal pain, with symptoms evolving over 6 weeks. Blood screening tests were normal except for raised aspartate aminotransferase (AST). Radiographs revealed thickened periosteum, widening of the diaphyses of long bones and lifted periosteum in mid-shaft of ulnae and right femur. Skeletal scintigraphy showed a high uptake of radionuclide at clinically affected and unaffected sites, suggestive of multifocal osteoblastic skeletal lesions. After repeated enquiries, his parents admitted to giving him massive doses of preformed vitamin A for over 3 months as 'health tablets'. Surprisingly, he did not have overt liver disease typically found with much smaller doses, although the dermal changes and musculoskeletal pathology were florid. He made a full clinical recovery within 2 months of cessation of vitamin A.

The authors describe a five year-old plump looking female child with poorly controlled type 1 diabetes mellitus, hepatomegaly and growth retardation. Contrary to the usual phenotype of thin emaciated appearance in type 1 diabetes mellitus, the presence of above features should make one suspect Mauriac syndrome. Since the basis of this syndrome is poor metabolic control, early recognition is important. The authors emphasize the importance of optimal glycaemic control in preventing complications associated with Mauriac syndrome.

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