The Chiari malformation type 1 (CM1) has been subject to a recent examination anatomically and clinically where caudal displacement of the hindbrain through the foramenmagnum more than 5mm. The advent of modern neuroimaging has increased not only the detection of the malformation but also our understanding of it. The aim of this study was to attempt and to understand the clinical presentation of the disease with respect to morphology on MRI. The study consisted of 36 patients who presented to our institute Andhra Medical College between January 2008 and December 2011, in whom a diagnosis of CM1 with syringomyelia. Incidence of CM1 with syringomyelia in the present study was observed in youngest patient were 15years old and the oldest being 45 years with an average of 30 years and the sex distribution in the present study was female predominance. Examination by CT-scan showed no hydrocephalous, Magnetic resonance imaging (MRI) showed CM1 with syringomeylia in all 36 patients. The location of the syrinx was cervical in 14 cases and 22 cases had cervico dorsal. The symptoms observed in the present study were ranging from 3 months to 2 years with an average period of 13.5 months. All the patients in this study population underwent small sub occipital craniectomy and foramenmagnum decompression. The findings from the present study conclude that, clinical presentations of patients with CM1 with or without syringomyelia can be extremely diverse and heterogenous. The advent of magnetic resonance imaging has revolution the mainly, chari1 in our understanding of disease pathogenesis and may help to elucidate the different presentations of the disease. This would help to elucidate the nature and pathogenesis of atypical presentations.